melphalan and Lymphatic-Diseases

We report three patients with AL amyloidosis manifesting as systemic lymphadenopathy, mainly in the cervical and supraclavicular regions. Histopathology of lymph nodes showed massive deposition of AL amyloid with no abnormal findings suggestive of lymphoproliferative disorders. Two of the patients were considered to be classifiable as primary systemic AL amyloidosis based on the presence of M-protein in serum and abnormal plasma cells or lymphoplasmacytoid cells in the bone marrow probably producing the precursor immunoglobulin, although no visceral organs were affected. The size of the involved lymph nodes in these two patients increased gradually, and one was treated with rituximab and VAD (vincristine, doxorubicin and dexamethasone) followed by high-dose melphalan with autologous peripheral blood stem cell transplantation (auto-PBSCT). The remaining patient showed no obvious change in the size of lymph nodes or detectable M-protein in serum. The prognosis of AL amyloidosis manifesting as lymphadenopathy is usually good as long as there are no hematological malignancies or rapid increases in the size of lymph nodes, but in cases of the systemic type, intensive chemotherapy, such as high-dose melphalan with auto-PBSCT, should be actively considered in order to avoid possible involvement of visceral organs.

Topics: Aged; Amyloid; Amyloidosis; Antineoplastic Agents, Alkylating; Diagnosis, Differential; Female; Humans; Lymph Nodes; Lymphatic Diseases; Male; Melphalan; Middle Aged; Peripheral Blood Stem Cell Transplantation; Transplantation, Autologous

After ilioinguinal radical lymph node dissection (RLND), the therapy for lymph fistulas constitutes a challenge. Risk factors for the genesis of lymph fistulas have not been sufficiently evaluated. We investigated possible factors that could influence the development of lymph fistulas in patients suffering from malignant melanoma after iloinguinal RLND.. The analysis was related to patients with intransit and lymphonodal metastasised malignant melanoma of the lower limb, who underwent RLND and isolated limb perfusion (ILP). Prospective data acquisition from patients undergoing ilioinguinal RLND and ILP in a one-step approach was performed. The association of lymph fistulas to risk factors was calculated using chi-squared, linear-by-linear test and ROC curves. As possible risk factors we investigated the presence of prior surgery and diabetes mellitus type II in the medical history, chemotherapeutics, patient age and the body mass index (BMI).. Postoperative lymph fistula occurred in 11 of 108 patients (10.2%). A significant association to lymph fistulas was found in BMI (30.2± 7.0 kg/m (2), p<0.02). Other parameters, such as prior surgery (82% vs. 71%), diabetes mellitus type II (9% vs. 11.7%), chemotherapeutics and patient age (mean 67.8 vs. 62.4 years) showed no influence.. Our results indicate that the incidence of lymph fistulas after RLND and ILP of malignant melanoma of the lower limb was associated with an increased BMI. Thus, for the prevention of lymph fistulae, an initially alternative wound-closure dressing like vacuum assisted closure (V.A.C.) dressing could be of clinical relevance for obese patients.

Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Body Mass Index; Chemotherapy, Adjuvant; Chemotherapy, Cancer, Regional Perfusion; Female; Fistula; Humans; Inguinal Canal; Leg; Lymph Node Excision; Lymphatic Diseases; Male; Melanoma; Melphalan; Middle Aged; Negative-Pressure Wound Therapy; Neoplasm Staging; Obesity; Postoperative Complications; Retrospective Studies; Risk Factors; Skin Neoplasms; Tumor Burden; Tumor Necrosis Factor-alpha

Pulmonary Langerhans histiocytosis (PLH) is a rare disease due to the accumulation of Langerhans cells at the level of the bronchioles. These dendritic immunocytes form granulomata and destroy the wall of the airway. We report a case of PLH developing at the same time as Hodgkin's lymphoma in a young woman who smoked tobacco and cannabis. We observed a complete remission of the PLH lesions parallel to the remission of the Hodgkin's lymphoma after chemotherapy, in the absence of any change in the consumption of tobacco and cannabis. This observation leads us to discuss the potential relationships between PLH on one hand, and smoking, the lymphoma and its treatment on the other.

Topics: Accidents, Traffic; Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Bronchioles; Carmustine; Cytarabine; Dacarbazine; Doxorubicin; Etoposide; Female; Histiocytosis, Langerhans-Cell; Hodgkin Disease; Humans; Ifosfamide; Incidental Findings; Lung Diseases; Lymphatic Diseases; Marijuana Smoking; Melphalan; Methylprednisolone; Mitoguazone; Remission Induction; Smoking; Tomography, X-Ray Computed; Vinblastine; Vindesine; Vinorelbine; Young Adult

A male patient with primary AL amyloidosis who had been suffering from systemic lymphadenopathy with IgMkappa-type M-proteinemia received two courses of VAD and high-dose melphalan with in vivo elimination of CD20(+) cells using rituximab followed by autologous peripheral blood stem cell transplantation. Four years after complete hematological remission he showed marked reduction in size of the amyloid-laden lymph nodes. Deposits of AL amyloid may regress from the tissue if the chemotherapy succeeds in persistent inhibition of the production of amyloidogenic immunoglobulin light chains.

Topics: Amyloidosis; Antineoplastic Agents, Alkylating; Humans; Immunoglobulin M; Lymph Nodes; Lymphatic Diseases; Male; Melphalan; Middle Aged; Radiography

A male patient with primary AL amyloidosis who had been suffering from systemic lymphadenopathy with IgMkappa-type M-proteinemia received two courses of VAD and high-dose melphalan with in vivo elimination of CD20(+) cells using rituximab followed by autologous peripheral blood stem cell transplantation. Four years after complete hematological remission he showed marked reduction in size of the amyloid-laden lymph nodes. Deposits of AL amyloid may regress from the tissue if the chemotherapy succeeds in persistent inhibition of the production of an amyloidogenic immunoglobulin light chain.

Topics: Amyloid; Amyloidosis; Antineoplastic Agents, Alkylating; Humans; Lymphatic Diseases; Male; Melphalan; Middle Aged

Lymphadenopathy associated with hemorrhage as a presenting feature of primary (AL) amyloidosis has not previously been described. We report two such cases one of whom had an acquired factor X and IX deficiency. The clinical presentations were characterized by sudden spontaneous enlargement of lymph nodes followed by partial regression. In both cases significant delay in diagnosis, and hence treatment, occurred due to the mode of presentation. One patient died with rapidly progressive disease but the other has had an excellent response to therapy with high-dose melphalan (HDM, 200 mg/m2) and peripheral blood stem cell rescue. AL amyloid should be considered in all patients presenting with hemorrhagic lymphadenopathy.

Topics: Adult; Amyloid; Amyloidosis; Diagnosis, Differential; Factor X Deficiency; Fatal Outcome; Hematopoietic Stem Cell Transplantation; Hemophilia B; Hemorrhage; Humans; Liver; Lymphatic Diseases; Male; Melphalan; Microscopy, Polarization; Middle Aged

One hundred and fifty-six patients with extremity melanomas of known level or thickness who were perfused prophylactically with l-phenylalanine mustard (1-PAM) between January 1974 and December 1978 were studied retrospectively to determine the effect of variation of drug dosage and temperature on regional toxicity and disease control. The median drug dosage of 1-PAM for 57 patients undergoing axillary perfusion was 0.85 mg/kg (range 0.48-1.0 mg/kg) and the median dosage was 1.2 mg/kg (range 0.59-1.69 mg/kg) for 99 patients undergoing iliac perfusions. Sixty-five percent of patients achieved a maximum skin temperature of between 101 degrees and 102 degrees F during perfusion. Determinate survival in the entire group was 93% at 5 years; 10% of patients developed positive regional nodes; and 2.5% developed local or intransit metastases. Based on analysis of other series of patients with extremity melanoma with equivalent Clark's level 5-year determinate survival might be expected to be between 65 and 80%. The expected incidence of nodal metastases should be 19.1%-24.0% and the incidence of local and intransit metastases should be 3-6%. While this series suggests a survival advantage for a series of extremity melanomas treated by regional chemotherapy when compared to other series treated by wide excision +/- regional node dissection, the results obtained were independent of dosage of drug administered or maximal temperature attained over the range studied. This suggests consideration be given to exploring other dose ranges of drugs and heat in an effort to achieve equivalent control with lower regional toxicity.

Topics: Adolescent; Adult; Aged; Arm; Chemotherapy, Cancer, Regional Perfusion; Child; Dose-Response Relationship, Drug; Female; Humans; Hyperthermia, Induced; Leg; Lymphatic Diseases; Lymphatic Metastasis; Male; Melanoma; Melphalan; Middle Aged; Skin Neoplasms

The incidence of a lymphoreticular system malignancy in patients with chronic lymphocytic leukemia (CLL), Richter's syndrome (RS), is 3.3 to 10.6%. In 89 cases in the literature, the second neoplasm was either reticulum cell sarcoma or large cell diffuse histiocytic lymphoma (DHL), and there were 61 cases of Hodgkin's disease (HD). In a few cases the lymphoma was simultaneously diagnosed, for other cases an association with preexisting CLL was reported. Appearance of lymphoma was associated with leukemia regression for only 4 patients with DHL and 3 with HD. We report one case of B-lymphocyte CLL with macroglobulinemia, treated with melphalan and prednisone, in which DHL developed and the hematologic and histologic signs of CLL and of paraproteinemia remissed. Such patients might constitute a subgroup or a variant of RS. Since the non-Hodgkin malignant lymphomas (NHML) are considered to be monoclonal neoplastic expansion of the B-cell or T-cell lymphocytes, and since in some cases it has been proved that the proliferative cell clone was the same as that of the initial lymphoproliferative disease, RS could be a dedifferentiation or a transformation of CLL, resulting in an aggressive clinical course. The inclusion in this syndrome of CLL cases associated with HD is still controversial. Many of these cases could be giant cell pleomorphic lymphomas, while, on the contrary, in typical cases this association might be merely fortuitous.

Topics: Blood Transfusion; Female; Hemosiderosis; Humans; Immunoglobulin M; Leukemia, Lymphoid; Lymph Nodes; Lymphatic Diseases; Melphalan; Middle Aged; Paraproteinemias; Prednisone; Syndrome

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.

Topics: Allopurinol; Anemia; B-Lymphocytes; Cryoglobulins; Female; Humans; Leukopenia; Lymphatic Diseases; Melphalan; Middle Aged; Paraproteinemias; Prednisone; Purpura, Hyperglobulinemic

A patient with immunoblastic lymphadenopathy (IL) had an unusual course of illness, with frequent episodes, over a 20-month period, of chills, fever, abdominal pain, hepatosplenomegaly and weight loss. The episodes were short-lived and many resolved spontaneously. Eventually generalized lymphadenopathy and profound monoclonal IgG gammopathy developed, with atypical mononuclear cells in the peripheral blood and increased numbers of plasmacytoid cells in the bone marrow. Lymph node biopsy revealed the morphologic triad typical of IL: proliferation immunoblasts, proliferation of small blood vessels and the deposit of an amorphous acidophillic material in the vascular walls and the interstitium. Up to October 1976 110 cases had been reported of this disorder, first described 3 years ago, which indicates that IL is not rare. Remissions have occurred spontaneously and after steroid therapy or chemotherapy or both. However, death has been reported in almost 50% of the cases, and the best approach to therapy remains to be determined.

Topics: Aged; Bone Marrow; Cell Nucleus; Cyclophosphamide; Endoplasmic Reticulum; Female; Humans; Immune System Diseases; Lymph Nodes; Lymphatic Diseases; Melphalan; Mitochondria; Plasma Cells; Prednisone

Topics: Chlorambucil; Cyclophosphamide; Dermatitis, Exfoliative; Humans; Keratoderma, Palmoplantar; Lymphatic Diseases; Lymphoma; Melphalan; Methotrexate; Nitrogen Mustard Compounds; Prednisone; Pruritus; Remission, Spontaneous; Syndrome; Time Factors; Triethylenemelamine; Vinblastine

Topics: Blood Protein Disorders; Blood Protein Electrophoresis; Cyclophosphamide; gamma-Globulins; Humans; Immunoelectrophoresis; Lymphatic Diseases; Melphalan; Multiple Myeloma; Plasmacytoma; Skeleton; Waldenstrom Macroglobulinemia

Topics: Adrenal Cortex Hormones; Adult; Blood Transfusion; Female; Humans; Leukemia; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Male; Melphalan; Middle Aged; Uracil; Vitamins

Topics: Chlorambucil; Cyclophosphamide; Genetic Diseases, X-Linked; Hodgkin Disease; Humans; Leukemia, Hairy Cell; Lymphatic Diseases; Melphalan; Nitrogen Mustard Compounds; Peptide Nucleic Acids; Prednisolone; Prednisone; Severe Combined Immunodeficiency; Thiotepa

Topics: Antineoplastic Agents; Genetic Diseases, X-Linked; Humans; Leukemia; Leukemia, Erythroblastic, Acute; Leukemia, Hairy Cell; Lymphatic Diseases; Lymphoma; Melphalan; Nitrogen Mustard Compounds; Severe Combined Immunodeficiency