melphalan and Leukemia--Plasma-Cell

Pleural effusion or ascites complicating plasmacytoma is rare and has a poor prognosis. A 70-year-old man was diagnosed as plasma cell leukemia and one course of ranimustine-vindesine, melphalan, and prednisolone followed by melphalan and prednisone (MP) maintained a very good partial response. After MP he was diagnosed to have pleural effusion and ascites as a complication of the plasmacytoma. Low-dose bortezomib caused disappearance of the malignant effusion. The malignant effusions recurred after the end of the second course of bortezomib. High-dose dexamethasone vincristine, doxorubicin, cyclophosphamide, and prednisone yielded no benefit, the patient died of Aspergillus pneumonia.

Topics: Aged; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Ascites; Boronic Acids; Bortezomib; Fatal Outcome; Humans; Leukemia, Plasma Cell; Male; Melphalan; Pleural Effusion, Malignant; Prednisone; Pyrazines; Treatment Failure

Plasma cell leukemia (PCL) is a rare lymphoproliferative disorder characterized by a malignant proliferation of plasma cells in blood and bone marrow. Treatment of primary PCL has been mostly disappointing. Three patients with primary PCL are described who received high-dose melphalan with autologous PBSC support after vincristine, doxorubicine and dexamethasone (VAD), high-dose cyclophosphamide, and etoposide, cisplatinum, dexamethasone and cytosine arabinoside (EDAP) courses. All patients were in CR post-transplantation. One patient relapsed after 3 months; the other patients are still in CR, after 14 and 26 months, respectively. These results in conjunction with data from the literature suggest that intensive chemotherapy for PCL is promising.

Topics: Antineoplastic Combined Chemotherapy Protocols; Cisplatin; Combined Modality Therapy; Cytarabine; Dexamethasone; Doxorubicin; Etoposide; Female; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Plasma Cell; Melphalan; Middle Aged; Paraproteins; Remission Induction; Treatment Outcome; Vincristine

Topics: Blood Coagulation; Blood Coagulation Disorders; Blood Coagulation Factors; Fibrinolysis; Humans; Leukemia, Plasma Cell; Melphalan; Multiple Myeloma; Paraproteinemias; Plasminogen; Plasminogen Activators

Primary plasma cell leukemia (PPCL) is a rare form of disease accounting for 1-2 percent of myelomas. Between September 1990 and November 2000, among 540 patients with myeloma studied, 24 fulfilled the criteria of PPCL (4.4 percent). We found high frequencies of female patients (62 percent), Bence Jones proteinuria (79 percent), anemia (88 percent), bleeding (54 percent), confusional syndrome (42 percent), weight loss (71 percent), hepatomegaly (25 percent), splenomegaly (21 percent), leukocytosis (62 percent), and thrombocytopenia (71 percent). High serum levels of creatinine, calcium, lactate dehydrogenase (LDH), and beta(2)-microglobulin were detected in 50 percent, 37 percent, 58 percent, and 71 percent, respectively. Four patients were treated with vincristine, melphalan, cyclophosphamide, prednisone, and adriamycin (VMCPA), 12 with vincristine, adriamycin, and dexamethasone (VAD), and 8 with M-80 (oral melphalan 80 mg/m(2) plus dexamethasone 40 mg/m(2)). There was a trend toward lower values of Karnofsky score (P=0.07) and higher values of LDH (P=0.2) in the VAD group. Other clinical characteristics were comparable among the three groups. Complete plus partial responses were achieved in one and six patients treated with VMCPA and M-80, respectively. All patients treated with VAD failed to respond to treatment. Patients receiving the M-80 regimen experienced higher platelet toxicity (P=0.05), vomiting (P<0.0003), and mucositis. Also, the need for red blood cell transfusions was higher in the M-80 group. Median overall survival was 60 days. Overall survival was better in patients achieving complete or partial response. In conclusion, our study illustrates that intermediate doses of melphalan plus dexamethasone are an effective chemotherapy regimen for this aggressive disease. Response to treatment is the only prognostic factor for survival in these patients.

Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Antineoplastic Agents, Alkylating; Antineoplastic Agents, Hormonal; Antineoplastic Agents, Phytogenic; Antineoplastic Combined Chemotherapy Protocols; Dexamethasone; Dose-Response Relationship, Drug; Doxorubicin; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Photochemotherapy; Survival Analysis; Treatment Outcome; Vincristine

Topics: Aged, 80 and over; Anemia, Hemolytic, Autoimmune; Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Bortezomib; Clonal Evolution; Clone Cells; Cyclophosphamide; Dexamethasone; Disease Progression; Female; Humans; Immunosuppressive Agents; Leukemia, Lymphocytic, Chronic, B-Cell; Leukemia, Plasma Cell; Lymphocytosis; Melphalan; Prednisolone; Rituximab

Topics: Adult; Female; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Myeloablative Agonists; Transplantation Conditioning; Transplantation, Homologous; Treatment Outcome; Whole-Body Irradiation

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Boronic Acids; Bortezomib; Chromosomes, Human, Pair 13; Chromosomes, Human, Pair 17; Dexamethasone; Disease Progression; Female; Genes, p53; Humans; In Situ Hybridization, Fluorescence; Lenalidomide; Leukemia, Plasma Cell; Male; Melphalan; Multiple Myeloma; Prednisone; Pyrazines; Sequence Deletion; Thalidomide; Treatment Outcome; Trisomy

The best therapeutic approach for primary plasma cell leukemia (PPCL) remains unknown so far. In very limited studies, the poor clinical outcome of this aggressive variant of multiple myeloma seemed to be ameliorated by the use of the proteasome inhibitor bortezomib. Aiming to provide more consolidated data, this multicenter retrospective survey focused on unselected and previously untreated PPCL patients who had received bortezomib as frontline therapy.. Twenty-nine patients with PPCL were collected. Bortezomib was given at standard doses and schedules, in various combinations with dexamethasone, thalidomide, doxorubicin, melphalan, prednisone, vincristine, and cyclophosphamide.. An overall response rate of 79% was observed, with 38% of at least very good partial remission. Grade 3-4 hematological, neurological, infectious, and renal toxic effects occurred in 20%, 21%, 16%, and 4% of patients, respectively. After a median follow-up of 24 months, 16 patients were alive (55%), 12 of whom were in remission phase and 4 relapsed. The best long-term results were achieved in patients who received stem-cell transplantation after bortezomib induction.. Bortezomib, used as initial therapy, is able to increase the percentage and the quality of responses in PPCL patients, producing a significant improvement of survival.

Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Boronic Acids; Bortezomib; Cyclophosphamide; Dexamethasone; Disease-Free Survival; Doxorubicin; Female; Humans; Kaplan-Meier Estimate; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisone; Pyrazines; Retrospective Studies; Thalidomide; Treatment Outcome; Vincristine

Topics: Antibodies, Monoclonal, Murine-Derived; Antineoplastic Agents; Antineoplastic Agents, Alkylating; Antineoplastic Combined Chemotherapy Protocols; Boronic Acids; Bortezomib; Cyclophosphamide; Dexamethasone; Glucocorticoids; Hematopoietic Stem Cell Transplantation; Humans; Lenalidomide; Leukemia, Plasma Cell; Melphalan; Pyrazines; Rituximab; Thalidomide

Topics: Aged; Antineoplastic Agents, Alkylating; Bone Marrow Cells; Diphosphonates; Drug Therapy, Combination; Fatal Outcome; Female; Humans; Imidazoles; Immunoglobulin A; Immunoglobulin E; Immunoglobulin kappa-Chains; Leukemia, Plasma Cell; Melphalan; Multiple Myeloma; Myeloma Proteins; Neoplasms, Second Primary; Prednisone; Zoledronic Acid

Topics: Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Boronic Acids; Bortezomib; Female; Humans; Leukemia, Plasma Cell; Melphalan; Prednisolone; Pyrazines; Remission Induction

Plasma cell leukemia is a rare, aggressive form of multiple myeloma. A 35-year-old male presented with backache, generalized weakness, and facial puffiness. His complete blood count showed anemia and a high WBC count with atypical cells on peripheral smear. Bone marrow examination showed more than 90% of atypical plasma cells, confirming a diagnosis of plasma cell leukemia. Patient also had azotemia, hypercalcemia, and hyperuricemia. The patient was started on chemotherapy along with supportive care. Patient improved dramatically and he was discharged on regular follow-up.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Humans; Leukemia, Plasma Cell; Male; Melphalan; Prednisolone; Thalidomide

A 69-year-old Japanese man was diagnosed as having primary plasma cell leukemia. His malignant plasma cells had a chromosomal translocation t(11;14)(q13;q32) that created overexpression of cyclin D1. Two courses of VAD (vincristine, doxorubicin, dexamethasone) therapy failed to achieve complete remission. Three subsequent courses of hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) therapy successfully induced remission with negative FISH test for t(11;14)(q13;q32). Thereafter, the patient received high-dose melphalan (125 mg/m(2)) followed by autologous peripheral blood stem cell transplantation. Cyclin D1 that was present prior to the high-dose chemotherapy, was no longer detected by qualitative PCR analysis. Despite complete cytogenetic remission, the disease relapsed 6 months later, and the patient eventually died 16 months following the diagnosis. Plasma cell leukemia is a rare hematological malignancy with a poor prognosis. The treatment has not been standardized yet. The present case suggested the effectiveness of the combination of hyper-CVAD and high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. Nevertheless, because of the short remission duration, intensification using tandem high-dose chemotherapy or maintenance using new agents such as bortezomib and thalidomide should be considered for improving the prognosis.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Dexamethasone; Doxorubicin; Humans; Leukemia, Plasma Cell; Male; Melphalan; Peripheral Blood Stem Cell Transplantation; Polymerase Chain Reaction; Remission Induction; Transplantation, Autologous; Treatment Failure; Vincristine

Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. We report a patient who presented with history of high grade fever, weakness, palpitations, loss of appetite, bone pains and mental confusion for twenty days. Initial evaluation revealed plasmacytosis with blood plasma cell count of 5184/cumm. His hemoglobin (Hb) was 11.3 gm/dl, platelets were 75000/cumm and total leucocyte count (TLC) was 21600/cumm (24% plasma cells). Bone marrow examination revealed >60% plasmablasts. Serum LDH was high at 3117 U/L and serum calcium was also elevated at 13.9 mg/dl. A diagnosis of PCL was made and the patient was started on treatment for hypercalcaemia with Melphalan/Prednisolone regime along with supportive care. Patient deteriorated very rapidly despite treatment and died on the eighth day. A detailed report of this case and a review of PCL is presented here.

Topics: Antineoplastic Agents, Alkylating; Drug Therapy, Combination; Fatal Outcome; Glucocorticoids; Humans; Leukemia, Plasma Cell; Leukocyte Count; Male; Melphalan; Middle Aged; Multiple Myeloma; Plasma Cells; Prednisolone

Topics: Acute Disease; Aged; Antineoplastic Combined Chemotherapy Protocols; Clone Cells; Combined Modality Therapy; Dexamethasone; Disease Progression; Doxorubicin; Fatal Outcome; Female; Gene Rearrangement, B-Lymphocyte, Heavy Chain; Humans; Leukemia, Plasma Cell; Leukemia, Radiation-Induced; Melphalan; Multiple Myeloma; Neoplasms, Second Primary; Neoplastic Stem Cells; Polymerase Chain Reaction; Prednisone; Radiotherapy; Vincristine

A 78-year-old woman was admitted to our hospital because of lumbago and appetite loss. Blood analysis revealed anemia, hypercalcemia and circulating plasma cells. Bone marrow aspiration showed an elevated ratio (43%) of plasma cells, which expressed CD38 in the absence of CD19 and CD56 expression. Spinal MR imaging revealed multiple compression fractures and suggested diffuse invasion of plasma cells into the spinal bodies. No M-protein was detectable in serum or urine by immunoelectrophoresis and immunofixation, but cytoplasmic M-protein (IgG-kappa) was detected by enzyme antibody staining. On the basis of the history and data, nonsecretory primary plasma cell leukemia was diagnosed. First, the patient was given modified VAD therapy (vincristine, doxorubicin, and prednisolone) and complete remission was obtained. Then MP therapy (melphalan and prednisolone) was instituted, and remission has since been maintained for 11 months. Like many other cases of primary plasma cell leukemia, this case suggests that CD56 may act as an adhesion molecule between neoplastic plasma cells and bone marrow stromal cells. Our experience with this exceedingly rare case suggests the superiority of combination chemotherapy as an induction therapy and the effectiveness of MP therapy as maintenance therapy for this disease.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Doxorubicin; Drug Administration Schedule; Female; Humans; Leukemia, Plasma Cell; Melphalan; Prednisolone; Prednisone; Vincristine

We report on a series of 26 patients diagnosed with primary (de novo) plasma cell (PC) leukemia (PCL) in whom we analyzed the clinicobiologic characteristics of the disease together with the immunophenotype, DNA cell content, proliferative index, and numeric chromosomal aberrations of the neoplastic PC, and compared them with 664 multiple myeloma (MM) patients at diagnosis. The median age, sex ratio, and bone lesion extension were similar, but PCL cases displayed a higher prevalence of clinical stage III, extramedullary involvement, and Bence Jones cases, with fewer IgA cases than for MM patients. In addition, according to several prognostic indicators (beta2-microglobulin serum level, proportion of S-phase PCs, proteinuria, calcium serum level, lactate dehydrogenase [LDH] and renal function), the incidence of adverse prognostic factors was significantly higher in PCL versus MM. Immunophenotypic expression was similar for CD38, CD138, CD2, CD3, CD16, CD10, CD13, and CD15, but PCL differed from MM in the expression of CD56, CD9 HLA-DR, CD117, and CD20 antigens. Twenty-two PCL cases were diploid and one was hypodiploid, while most MM cases (57%) showed DNA hyperdiploidy. With the fluorescent in situ hydridization (FISH) technique, 12 of 13 PCL cases displayed the numeric aberrations, -13 (86%), +/-1 (57%), +18 (43%), and -X in women (25%), but they lacked several numeric aberrations usually found in MM such as +3, +6, +9, +11, and +15. PCL cases had a lower overall response to therapy than MM cases (38% v 63%, P =.01332). Among PCL patients, a trend for a worse response was observed in cases treated with melphalan and prednisone (MP) versus polychemotherapy. Overall survival was significantly worse in PCL versus MM patients (8 v 36 months, P <.0001), but it was significantly better in PCL patients treated with polychemotherapy versus MP (18 v 3 months, P =.0137). By contrast, MM patients did not show significant differences in overall survival according to the treatment used, MP or polychemotherapy. Ten variables seemed to predict survival in PCL patients, but only the beta2-microglobulin level and S-phase PCs retained an independent value in multivariate analysis. In summary, our study illustrates that PCs from PCL display singular phenotypic, DNA cell content, and cytogenetic characteristics that lead to a different disease evolution versus MM.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Chromosome Aberrations; Female; Humans; Immunophenotyping; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Mitotic Index; Multiple Myeloma; Neoplasm Proteins; Ploidies; Prednisone; Prognosis; Survival Rate; Treatment Outcome

Primary plasma cell leukemia (PCL) is a rare form of plasma cell neoplasm with a poor prognosis. Conventional melphalan-based treatments have been most disappointing. We report the case of a 62-year-old man with a primary form of PCL treated with VAD combination achieving an objective response, and who received high-dose melphalan and autologous peripheral blood stem cell (PBSC) transplantation followed by interferon-alpha. During the remission time, lasting for 3 years, an infiltration by large granular lymphocytes (LGL) was noted in peripheral blood. However, when the number of LGL declined, a bone marrow relapse was observed. The treatment for PCL and the possible role of these LGL on tumor cell control after autologous PBSC transplantation are discussed.

Topics: Antineoplastic Combined Chemotherapy Protocols; Cytarabine; Dexamethasone; Hematopoietic Stem Cell Transplantation; Humans; Interferon-alpha; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Remission Induction; Survivors; Treatment Outcome; Vincristine

Nine patients were diagnosed with plasma cell leukemia (PCL) from 1982-1995 at our hospital. Seven patients had primary PCL and the other two patients a secondary from. In this study the clinical and analytical features are reported, as well as the therapy and response obtained in these patients. Also, the karyotype findings in bone marrow of four of these patients are reported. At diagnosis, the most common symptom was bone pain which was associated with osteolytic lesions or diffuse bone demineralization. Analytical features were similar to those reported in other series of patients with PCL. Different therapeutical regimens were used, and VAD was the most commonly employed. Two patients underwent consolidation therapy with autologous transplantation of hemopoietic stem cells. The mean survival time was 5.5 months. Although PCL prognosis associated with chemotherapy is still poor, myeloablative therapy with hemopoietic support can increase the survival length in these patients.

Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Dexamethasone; Doxorubicin; Female; Humans; Immunoglobulin kappa-Chains; Immunoglobulin lambda-Chains; Leukemia, Plasma Cell; Male; Melphalan; Prednisone; Vincristine

Topics: Acute Disease; Adult; Anti-Bacterial Agents; Antineoplastic Combined Chemotherapy Protocols; Daunorubicin; Diuretics; Drug Therapy, Combination; Female; Humans; Leukemia, Plasma Cell; Melphalan; Prednisolone; Time Factors; Vasodilator Agents; Vincristine

We report a case of de novo plasma cell leukemia, resistant to standard VMD (vincristine, mitoxantrone, dexamethasone) and CVP (cyclophosphamide, vincristine and prednisone) protocols, treated with a chemotherapy intensification regimen (high-dose cyclophosphamide, modified EDAP, Dexa-BEAM) and peripheral blood stem cell transplantation, performed using fractionated total body irradiation and high dose melphalan. The patient is currently alive and well, in very good partial remission 12 months after transplant and 22 months after diagnosis, disclosing a significant proportion of bone marrow and peripheral blood CD3+, CD8+, CD57+, HLA-Dr+ large granular lymphocytes with cytotoxic activity against neoplastic plasma cells.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Carmustine; Cisplatin; Cyclophosphamide; Cytarabine; Dexamethasone; Etoposide; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Plasma Cell; Lymphocyte Count; Male; Melphalan; Mitoxantrone; Prednisone; Remission Induction; T-Lymphocyte Subsets; Transplantation Conditioning; Vincristine; Whole-Body Irradiation

A 76-year-old female was diagnosed as having primary plasma cell leukemia (PCL), based on abundant atypical plasma cells in the circulation and bone marrow, monoclonal kappa light chain in the serum and urine, the immunophenotype of the plasma cells and the lack of preceding multiple myeloma. The patient was treated with melphalan and prednisolone (MP), and complete remission (CR) was achieved; this was maintained for 28 months. The duration of CR in this patient appears to be the longest of those reported in well-documented primary PCL patients who have been treated with MP chemotherapy.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Leukemia, Plasma Cell; Melphalan; Prednisolone; Remission Induction

Topics: Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Dexamethasone; Doxorubicin; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisolone; Vincristine

Topics: Antineoplastic Combined Chemotherapy Protocols; Doxorubicin; Drug Administration Schedule; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisolone; Remission Induction; Vincristine

A 69-year-old woman with IgE/kappa plasma cell leukemia (PCL) was treated with a sequential combination of VAD (vincristine, doxorubicin, dexamethasone) and MP (melphalan, prednisolone) followed by interferon-alpha (IFN alpha). A complete remission was achieved for 14 months. Maintenance therapy with IFN alpha has continued for an additional 10 months. IgE PCL is extremely rare. The biological characteristics of the myeloma cells, including surface marker, adhesion molecule, karyotype, DNA analysis, and the response to various cytokines, are presented.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Dexamethasone; Doxorubicin; Female; Humans; Immunoglobulin E; Interferon-alpha; Leukemia, Plasma Cell; Melphalan; Prednisolone; Vincristine

A 29-year-old woman, with a slightly elevated temperature for 3 weeks, increasing dyspnoea at rest, markedly reduced general condition and in heart failure, was found to have a leucocytosis of 100,000/microliters, anaemia (haemoglobin 6.3 g/dl) and thrombocytopenia (41,000/microliters). There were 62% plasma cells in the blood smear. Immunoelectrophoresis of serum and urine revealed kappa-light chains and immunocytology demonstrated IgG-kappa. There was no radiological evidence of osteolysis, while ultrasound examination showed multiple abdominal lymphomas and marked hepatosplenomegaly. Bone marrow smear showed a 90% infiltration of plasma cells. High-dosage melphalan treatment (single intravenous injection of 140 mg/m2) resulted in complete remission after myelodepression over several weeks. Two extramedullary recurrences 5 and 12 months after the diagnosis had been made were successfully treated with high-dosage melphalan, but it was associated with severe and long-lasting myelodepression. Septicaemia with renal and hepatic failure developed and the patient died 6 weeks after the third course of high-dosage melphalan, 14 months after the diagnosis.

Topics: Acute Disease; Adult; Bone Marrow Examination; Cell Count; Female; Humans; Immunoglobulin Light Chains; Leukemia, Plasma Cell; Leukopenia; Melphalan; Plasma Cells; Thrombocytopenia

The levels of DNA adducts formed in peripheral blood mononuclear cells of 13 patients undergoing high-dose melphalan therapy were determined 0-24 h after drug administration using a modification of a previously described immunoassay. This assay was validated for DNA extracted from drug-treated cells. Adduct levels in normal mononuclear blood cells 1 h after drug administration correlated well (r = 0.846) with drug dose (expressed as mg/m2) and with area under the curve for plasma levels of melphalan during the first h (r = 0.842). 1 patient sustained a high degree of toxic side-effects from the melphalan treatment and showed a high level of adducts. Plasma cell leukaemia tumour cells from another patient showed a level of adducts approximately six times higher than those in the normal blood cells of the other patients. The levels of DNA adducts in normal peripheral blood mononuclear cells did not change markedly between 1 and 24 h after drug administration.

Topics: Alkylation; Cross-Linking Reagents; DNA; Dose-Response Relationship, Drug; Enzyme-Linked Immunosorbent Assay; Humans; Leukemia, Plasma Cell; Leukocytes, Mononuclear; Melphalan; Multiple Myeloma; Time Factors

A case of non-secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Bone pain was the initial clinical manifestation. Laboratory analysis showed 20% of circulating immature plasma cells. Despite the presence of osteolytic lesions, no M-component could be demonstrated in serum protein electrophoresis, and serum and urine immunoelectrophoresis. Bone marrow aspirate demonstrated an 83% infiltration of plasma cells showing various degrees of immaturity. Immunofluorescence with monoclonal antisera demonstrated intracytoplasmic kappa light chains in a high percentage of plasma cells. Immature plasma cells without cellular capacity to synthesize and excrete complete immunoglobulins could be more aggressive, leading to an initial leukaemic process. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features, and response to treatment of this extremely rare association are reviewed.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Carmustine; Cyclophosphamide; Female; Humans; Leukemia, Plasma Cell; Melphalan; Multiple Myeloma; Prednisone; Vincristine

Monoclonal gammopathy of type IgG-lambda (IgG concentration 27.8 g/l) was discovered by chance in a 66-year-old woman with aortic and mitral valve disease. The patient declined any further diagnostic procedures. Three months later she experienced severe pain in the lumbar spine and developed decompensated cardiac failure with pulmonary and ankle edema. The IgG concentration had risen to 50.5 g/l. Echocardiography showed a large pericardial effusion and 600 ml of bloodstained fluid containing numerous plasma cells was aspirated (total protein 81.8 g/l, gamma-globulin 38.9%). Iliac crest biopsy showed diffuse infiltration with polymorphic plasma cells, but the differential count in peripheral blood was unremarkable. Multiple myeloma of Stage IIa was diagnosed and she was given cytostatic therapy with 17.5 mg melphalan and 112 mg methylprednisolone daily by mouth (for 4 days at intervals of 6 weeks). Though at first the IgG concentration fell, it later rose again. The beta 2-microglobulin level was raised at 30 mg/l. After three cycles of chemotherapy the patient complained of severe pain in the hips and thighs. The blood film now showed numerous, predominantly immature plasma cells. A few days later, having been ill for four months in all, she died, showing all the signs and symptoms of plasma cell leukaemia.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Aortic Valve Insufficiency; Drug Therapy, Combination; Female; Humans; Immunoglobulin lambda-Chains; Leukemia, Plasma Cell; Melphalan; Methylprednisolone; Mitral Valve Stenosis; Multiple Myeloma; Pericardial Effusion; Plasma Cells

Clinical findings and response to treatment in four cases with plasma cell leukemia (PCL) out of 152 patients of multiple myeloma diagnosed at the Hospital La Paz from 1969 to 1988 are studied. Three of the four plasma cell leukemia cases presented a primary form, and one a secondary form. Our cases had a lower incidence of lymphadenopathy and splenomegaly than reported in previous series. The incidence of serum M band in PCL was similar to that found in multiple myeloma. The four patients received combination chemotherapy; one of them attained PR lasting for 2 months, and the remaining three failed to respond to similar therapy. The mean duration of survival was less than 8 months. Current treatments are reviewed.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Doxorubicin; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisolone; Prednisone; Remission Induction; Teniposide

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Transplantation; Combined Modality Therapy; Cyclophosphamide; Humans; Leukemia, Plasma Cell; Male; Melphalan; Transplantation, Homologous; Whole-Body Irradiation

Topics: Antineoplastic Combined Chemotherapy Protocols; Carmustine; Cyclophosphamide; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisone; Remission Induction; Time Factors; Vincristine

In the majority of cases plasma cell leukaemia is a rapidly fatal disease with a mean survival time of five months. There have been reports of increased survival using various regimens of chemotherapy although most cases eventually relapse. We describe a patient with primary plasma cell leukaemia who responded to a combination of oral melphalan and prednisolone with control of the disease in the bone marrow but relapsed with extramedullary disease in the central nervous system and testes, and died 22 months after diagnosis. Melphalan poorly penetrates the central nervous system and its testicular penetration is unknown.

Topics: Administration, Oral; Antineoplastic Combined Chemotherapy Protocols; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Prednisolone; Prognosis; Time Factors

Topics: Antineoplastic Combined Chemotherapy Protocols; Carmustine; Cyclophosphamide; Female; Humans; Leukemia, Plasma Cell; Melphalan; Middle Aged; Prednisone; Remission Induction; Vincristine

Two patients with primary plasma cell leukaemia who achieved complete remission are reported. They were treated with induction therapy consisting of a multipeptide derivative of sarcolysin, Peptichemio, given intravenously, combined with vincristine and/or prednisone, followed by conventional melphalan-prednisone therapy. 5-7 months following the beginning of therapy, both patients attained a complete remission which lasted 23 and 6 months; second remission was not achieved. Survival from starting therapy was 57 and 16 months respectively. These cases indicate that intravenous alkylating agents can induce a complete remission in plasma cell leukaemia similar to that achieved in other acute leukaemias.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Drug Evaluation; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Peptichemio; Prednisone; Time Factors; Vincristine

The case of a 74-year-old female with an IgD kappa plasma cell leukemia is presented. In contrast to the majority of the cases in the literature, this patient responded rather well to melphalan and prednisolone and survived for 20 months after starting treatment. Another remarkable feature was the simultaneous occurrence of a double uterine neoplasm, a malignant mixed Müllerian tumor of the homologous variety (also called carcinosarcoma).

Topics: Adnexa Uteri; Aged; Allopurinol; Ampicillin; Carcinosarcoma; Female; Gentamicins; Humans; Hysterectomy; Immunoglobulin D; Immunoglobulin kappa-Chains; Leukemia, Plasma Cell; Melphalan; Pneumonia; Prednisolone; Uterine Neoplasms

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Ethamsylate; Female; Humans; Hydroxyurea; Leukemia, Plasma Cell; Melphalan; Prednisone; Time Factors

1 previously untreated patient with plasma-cell leukaemia and 8 patients with myeloma (4 previously untreated) were treated with high-dose melphalan 100-140 mg/m2 iv. All responded to treatment. 3 of the 5 previously untreated patients achieved biochemical and bone-marrow complete remissions.

Topics: Adult; Age Factors; Bence Jones Protein; Bone Marrow Transplantation; Female; Humans; Injections, Intravenous; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Multiple Myeloma; Neutropenia; Pain Management; Transplantation, Autologous

Three patients with plasma cell leukemia are reported. Two of them has a previous history of myeloma; the third one started with a plasma cell leukemia. Diagnosis was made from the required presence of 20% plasma cells in the peripheral blood. In all 3 cases, bone marrow aspiration and peripheral blood showed plasma cells strongly positive for acid phosphatase and alpha-naphthyl acetate esterase, and negative for periodic acid-Schiff. The first patient was treated with a polychemotherapy regimen that included vincristine, cyclophosphamide, chlorambucil and prednisone, and the second patient with melphalan and prednisone; the third one, who started with plasma cell leukemia, received total body irradiation at the dose of 600 rad. The results of the therapy and survival time, which was never more than 3 months, are in accord with other reports in the literature.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Chlorambucil; Cyclophosphamide; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Multiple Myeloma; Prednisone; Time Factors; Vincristine; Whole-Body Irradiation

Fifteen patients with multiple myeloma, two of whom had plasma cell leukemia, were treated between May 1974 and December 1978. Peptichemio was administered intravenously at doses of 40-80 mg/48 h, courses including 4-17 administrations in association with moderate doses of prednisone (15-50 mg/day) and androstanes at high dosages (250 mg weekly). In two patients PTC was associated with vincristine (VCR) administered on the first day of the course. Eight patients were previously untreated, four had been resistant to melphalan (MPH) and/or cyclophosphamide (CTX), and three had been treated irregularly with one or both of these alkylating agents. The criteria of response to therapy are reported. Out of a total of 15 PTC courses administered we obtained 13 responses, eight complete and five partial; no response was achieved in the other two patients. In the four patients who were resistant to MPH and/or CTX we obtained three responses, which were maintained with the same alkylating agent to which they had been resistant previously. The time needed to obtain a response in 90% of the patients was 6 weeks. Peptichemio was shown to be effective in patients in an advanced stage of the disease, in patients with light-chain myeloma and in those with plasma cell leukemia. The association of VCR potentiated the antitumor effect, but also increased the myelotoxicity. The PTC treatment was well tolerated. It is suggested that PTC be used in induction treatment of myelomatosis and in patients resistant to traditional alkylating agents.

Topics: Adult; Aged; Alkylating Agents; Antineoplastic Agents; Blood Proteins; Cyclophosphamide; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Multiple Myeloma; Peptichemio

Topics: Aged; Drug Therapy, Combination; Female; Fructose; Humans; Immunoglobulin E; Indomethacin; Leukemia, Plasma Cell; Melphalan; Multiple Myeloma; Potassium Chloride; Prednisolone; Prednisone

Topics: Antineoplastic Agents; Carmustine; Cyclophosphamide; Cytarabine; Humans; Immunoglobulins; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Plasma Cells; Protein Biosynthesis; Vincristine

Fifteen patients presenting with plasma cell leukemia (PCL) are reported in detail. The clinicopathologic features of PCL differ from typical myeloma and resemble those of acute leukemia: patients with PCL have less bone disease but a much higher incidence of organomegaly and tissue infiltration as well as diffuse marrow involvement and more pronounced pancytopenia. One of the reported patients developed meningeal plasma cell leukemia and is reported in detail. Cytomorphologic assessment of PCL cells showed nuclear immaturity and obvious nuclear/cytoplasmic asynchrony. Despite the use of cytotoxic agents known to be effective in myeloma, the prognosis in PCL is poor, and the median survival of the reported patients was only 2 mo.

Topics: Adrenal Cortex Hormones; Adult; Aged; Cyclophosphamide; Female; Humans; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged

A 76-year-old man with plasma cell leukemia was treated with L-phenylalanine mustard and prednisone (CLGB 7461). There was good partial remission of the plasma cell disease characterized by disappearance of the plasma cells in the peripheral blood, reduction of plasma cells in the marrow aspirates to less than 5% of the nucleated hematopoietic cells, a reduction in the serum monoclonal IgG from 7.6 to 2 gms/100 ml, and the disappearance of urinary monoclonal IgG, Bence-Jones protein and a complex of gamma-chain fragment and beta2-microglobulins. There was also a marked improvement in the renal function and a decrease in the proteinuria from 4+ to 1+. The patient relapsed after more than 8 months of response and failed to respond to subsequent treatment with cytoxan and cytosine arabinoside. However, the efficacy of standard myeloma therapy was clearly apparent in this case of plasma cell leukemia.

Topics: Aged; Humans; Leukemia, Plasma Cell; Male; Melphalan; Multiple Myeloma; Prednisone

The peripheral blood cells of a patient with acute plasma cell leukemia were examined with transmission (TEM) and scanning (SEM) electron microscopes. The TEM features of the immature plasma cells comprised lobulated and irregulary shaped nuclei, with scanty heterochromating and bizarre nucleoli, parallel arrays of endoplasmic reticulum, cytoplasmic fibrils and numerous polymorphic mitochondria. SEM examination of the cells showed long, thin irregular ruffles, or round blebs on the cell surface, with appearance different from this observed on other types of leukemia. A remarkable clinical and hematological remission was achieved with administration of melphalan and steroids.

Topics: Acute Disease; Cell Nucleolus; Cell Nucleus; Endoplasmic Reticulum; Humans; Leukemia, Plasma Cell; Male; Melphalan; Microscopy, Electron; Microscopy, Electron, Scanning; Middle Aged; Mitochondria; Plasma Cells; Prednisone; Remission, Spontaneous

Topics: Adenocarcinoma; Female; Humans; Leukemia, Plasma Cell; Melphalan; Middle Aged; Neoplasms, Multiple Primary; Ovarian Neoplasms

Rapidly fatal acute myelomonocytic leukemia developed in five patients with multiple myeloma who were treated with melphalan for 28 to 54 months. In each patient, multiple myeloma responded to therapy and progress was satisfactory until the development of acute leukemia. At postmortem examination, leukemic infiltration of organs was seen, and there was little or no evidence of myeloma. Consideration of these cases and a review of the literature suggest that these circumstances represent the development of acute myelomonocytic leukemia rather than plasma cell leukemia; there also appears to be an increased incidence of acute leukemia in multiple myeloma, probably related to the alkylating agent.

Topics: Acute Disease; Aged; Bone Marrow; Bone Marrow Cells; Bone Marrow Examination; Bone Neoplasms; Hemoglobins; Humans; Leukemia, Myeloid; Leukemia, Plasma Cell; Leukocyte Count; Male; Melphalan; Microscopy, Electron; Middle Aged; Multiple Myeloma; Plasma Cells; Prednisone; Testosterone; Time Factors

Topics: Aged; Drug Therapy, Combination; Female; Humans; Immunoglobulin A; Immunoglobulin G; Leukemia, Plasma Cell; Male; Melphalan; Middle Aged; Paraproteins; Prednisone

Topics: Acid Phosphatase; Cyclophosphamide; Cytarabine; Drug Therapy, Combination; Fluorescent Antibody Technique; Glucuronidase; Histocytochemistry; Humans; Immunoelectrophoresis; Immunoglobulin Fragments; Karyotyping; Leukemia, Plasma Cell; Male; Melphalan; Microscopy, Electron; Microscopy, Fluorescence; Middle Aged; Plasma Cells; Prednisone; Prognosis; Remission, Spontaneous; Staining and Labeling; Vincristine

Topics: Adult; Aged; Bone Marrow Cells; Humans; Leukemia, Plasma Cell; Male; Melphalan; Microscopy, Electron; Plasma Cells

Topics: Aged; Antineoplastic Agents; Female; Humans; Kidney Failure, Chronic; Leukemia, Plasma Cell; Male; Melphalan; Microscopy, Electron; Microscopy, Fluorescence; Microscopy, Phase-Contrast; Middle Aged; Neoplastic Cells, Circulating; Prednisolone

Topics: Agammaglobulinemia; Aged; Anti-Bacterial Agents; Bence Jones Protein; Blood Cells; Blood Transfusion; Bone Marrow Cells; Humans; Leukemia, Plasma Cell; Male; Melphalan; Microscopy, Electron; Prednisone

Topics: Blood Protein Electrophoresis; Blood Proteins; Creatinine; Cyclophosphamide; Cytosine Nucleotides; Humans; Immunoglobulin G; Leukemia, Plasma Cell; Melphalan; Multiple Myeloma; Polynucleotides; Ribonucleases; RNA

Topics: Bence Jones Protein; Blood Protein Electrophoresis; Bone Marrow Examination; Diagnosis, Differential; Female; gamma-Globulins; Humans; Infarction; Leukemia, Plasma Cell; Leukocytosis; Melphalan; Middle Aged; Multiple Myeloma; Neoplasm Metastasis; Plasma Cells; Prednisone; Spleen; Splenic Neoplasms; Splenic Rupture; Subarachnoid Hemorrhage; Thrombocytopenia