mercaptopurine and Histiocytosis--Langerhans-Cell

Topics: Antigens, CD1; Biomarkers; Child; Diagnosis, Differential; Edema; Female; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Mercaptopurine; Orbital Diseases; S100 Proteins; Tomography, X-Ray Computed

Langerhans cell histiocytosis (LCH), which has unknown pathogenesis, can manifest as many kinds of signs and symptoms at any age. Although its genetic background has not been exactly identified, the familial clustering of this disease has been described in some reports. It is very uncommon, however, in siblings who are not monozygotic or dizygotic twins. Reported herein is a case of LCH in non-twin siblings (younger sister and elder brother) who were diagnosed at 3.3 and 14.5 years of age, respectively, and successfully treated with chemotherapy, with BRAF V600E mutation status, and a brief review of the literature.

Topics: Adolescent; Alleles; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Child, Preschool; Diagnosis, Differential; DNA Mutational Analysis; Female; Histiocytosis, Langerhans-Cell; Humans; Ilium; Liver Function Tests; Magnetic Resonance Imaging; Male; Mercaptopurine; Methotrexate; Orbit; Pedigree; Polymerase Chain Reaction; Prednisolone; Proto-Oncogene Proteins B-raf; Ribs; Vinblastine

Adult onset Langerhans cell histiocytosis (LCH) is a rare disorder. Its clinical features have been well described in children, however remain poorly defined in adults. Optimal treatment strategy is still under debate. We have encountered two cases of adult onset LCH, which obtained a durable disease control by combination chemotherapy using prednisone, vinblastine and 6-mercaptopurine. Herein, we report their clinical features together with a review of the current literature.

Topics: Adult; Age of Onset; Antineoplastic Combined Chemotherapy Protocols; Female; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Male; Mercaptopurine; Prednisone; Tomography, X-Ray Computed; Treatment Outcome; Vinblastine

Langerhans cell histiocytosis results from the abnormal accumulation of a class of dendritic cells normally found in the skin, which proliferate in many organ systems along with lymphocytes, macrophages and eosinophils. Standard therapy for Langerhans cell histiocytosis includes vinblastine and prednisone with or without methotrexate and mercaptopurine, depending on the extent of disease. Effective therapies for patients unresponsive to the above include cytosine arabinoside and cladribine. Thalidomide has proven useful for patients with Langerhans cell histiocytosis of the skin and/or bone. Emerging therapies include the use of monoclonal antibodies against the CD1a or CD52 epitopes found on Langerhans cells. Specific therapies directed against the cytokines that are apparently critical to the abnormal proliferation have not yet been defined.

Topics: Anti-Inflammatory Agents; Antimetabolites; Brain Diseases; Cell Proliferation; Cladribine; Cytarabine; Drug Therapy, Combination; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Mercaptopurine; Practice Guidelines as Topic; Prednisone; Randomized Controlled Trials as Topic; Risk Factors; Secondary Prevention; Thalidomide; Vinblastine

A 2-month-old girl presented for treatment with a diffuse rash, interstitial pneumonia, otorrhea, and lymphadenopathy. Skin biopsy confirmed Langerhans cell histocytosis by electron microscopy. After receiving multiple courses of chemotherapy, only marginal improvement was achieved, with progressive marrow and liver involvement. The decision was made to pursue a human leukocyte antigen-identical unrelated cord blood transplantation. Two years after transplant, the bone marrow was clear of Langerhans cell histocytosis and 100% donor engraftment. The poor prognosis of patients with an inadequate response to therapy and the presence of organ dysfunction (marrow and liver) substantiated the decision to pursue an unrelated cord blood transplantation.

Topics: Bone Marrow; Cladribine; Combined Modality Therapy; Cyclosporine; Cytarabine; Doxorubicin; Drug Therapy, Combination; Etoposide; Female; Fetal Blood; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Histiocytosis, Langerhans-Cell; Humans; Infant, Newborn; Mercaptopurine; Methotrexate; Methylprednisolone; Prednisone; Remission Induction; Transplantation, Homologous; Vinblastine

The authors describe a patient successfully treated with unrelated cord blood transplantation (CBT) for chemotherapy-resistant progressive Langerhans cell histiocytosis (LCH). An 8-month-old boy had LCH diagnosed based on the histologic examination of skin lesions. Despite intensive chemotherapy and immunotherapy, the disease was progressive, with organ dysfunction. He received unrelated CBT after a conditioning regimen consisting of total body irradiation, etoposide, and melphalan. He was in complete remission 12 months after the transplantation. The authors suggest that CBT could be considered in the treatment of patients with chemotherapy-resistant progressive LCH, especially if there are no available human leukocyte antigen-matched family donors.

Topics: Combined Modality Therapy; Cyclophosphamide; Cytarabine; Disease Progression; Doxorubicin; Drug Resistance; Drug Therapy, Combination; Etoposide; Fetal Blood; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Histiocytosis, Langerhans-Cell; Humans; Infant; Male; Mercaptopurine; Methotrexate; Methylprednisolone; Mitoxantrone; Prednisolone; Remission Induction; Transplantation Conditioning; Transplantation, Homologous; Vincristine

Langerhans cell histiocytosis (LCH)-III tested risk-adjusted, intensified, longer treatment of multisystem LCH (MS-LCH), for which optimal therapy has been elusive. Stratified by risk organ involvement (high [RO+] or low [RO-] risk groups), > 400 patients were randomized. RO+ patients received 1 to 2 six-week courses of vinblastine+prednisone (Arm A) or vinblastine + prednisone + methotrexate (Arm B). Response triggered milder continuation therapy with the same combinations, plus 6-mercaptopurine, for 12 months total treatment. 6/12-week response rates (mean, 71%) and 5-year survival (84%) and reactivation rates (27%) were similar in both arms. Notably, historical comparisons revealed survival superior to that of identically stratified RO+ patients treated for 6 months in predecessor trials LCH-I (62%) or LCH-II (69%, P < .001), and lower 5-year reactivation rates than in LCH-I (55%) or LCH-II (44%, P < .001). RO- patients received vinblastine+prednisone throughout. Response by 6 weeks triggered randomization to 6 or 12 months total treatment. Significantly lower 5-year reactivation rates characterized the 12-month Arm D (37%) compared with 6-month Arm C (54%, P = .03) or to 6-month schedules in LCH-I (52%) and LCH-II (48%, P < .001). Thus, prolonging treatment decreased RO- patient reactivations in LCH-III, and although methotrexate added no benefit, RO+ patient survival and reactivation rates have substantially improved in the 3 sequential trials. (Trial No. NCT00276757 www.ClinicalTrials.gov).

Topics: Adolescent; Child; Child, Preschool; Drug Therapy, Combination; Female; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Infant; Infant, Newborn; Kaplan-Meier Estimate; Male; Mercaptopurine; Methotrexate; Prednisone; Recurrence; Vinblastine

Treatment of Langerhans cell histiocytosis (LCH) remains problematic. To test the hypothesis that rapid initiation and long-term continuation of chemotherapy can improve survival and reduce recurrence and late consequences of disseminated LCH, we have completed a prospective clinical trial (DAL HX-83). One hundred six newly diagnosed patients were stratified into three risk groups (A: multifocal bone disease [n = 28]; B: soft tissue involvement without organ dysfunction [n = 57]; C: organ dysfunction [n = 21]). All patients received an identical initial 6-week treatment (etoposide [VP-16], prednisone, and vinblastine), and continuation treatment for 1 year, slightly adapted according to stratification at diagnosis. It included oral 6-mercaptopurine and eight pulses of vinblastine and prednisone for all patients, plus VP-16 in group B and VP-16 and methotrexate in group C. Eighty-nine percent and 91% of patients in groups A and B and 67% of the most severely affected group C, achieved complete resolution of disease. The speed of resolution was rapid (median 4 months) and independent of disease severity. The frequency of recurrence after initial resolution was low (12%, 23%, and 42% in groups A, B and C); overall fully 77% of patients have remained free of recurrence. Permanent consequences developed after diagnosis in 20% of the patients. Diabetes insipidus after initiation of treatment occurred in only 10% of patients. Mortality (9%) was limited to patients of groups B (two patients) and C (eight patients). Finally, among the 106 patients treated by DAL HX-83 none have developed a malignancy (median follow-up 6 years, 9 months). The shorter duration of active disease, low rate of recurrence and permanent consequences, and improved survival among patients with poor prognosis support the strategy of rapid initiation of a predefined prolonged treatment upon the diagnosis of disseminated LCH.

Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Diabetes Insipidus; Etoposide; Female; Histiocytosis, Langerhans-Cell; Humans; Infant; Infant, Newborn; Male; Mercaptopurine; Methotrexate; Prednisone; Prognosis; Prospective Studies; Recurrence; Remission Induction; Severity of Illness Index; Survival Analysis; Treatment Outcome; Vinblastine

Liver biopsies were done on 20 patients with histiocytosis X (HX) as part of pretreatment evaluation prior to entry on two Childrens Cancer Study Group protocols. Seventeen patients had hepatomegaly, and seven had one or more abnormal laboratory parameters using Lahey's criteria for liver dysfunction. Nineteen of 20 specimens showed various abnormalities of the portal triads. A single biopsy revealed normal liver. Among the changes were triaditis, bile duct proliferation, variable fibrosis with histiocytic infiltrates, and cirrhosis. One patient had typical granulomas of HX within the liver parenchyma in addition to portal triaditis. Patients with larger livers and dysfunction tended to show more marked histologic abnormalities in the portal triads. However, correlations among liver size, function, and pathology showed considerable overlap. Early death among these patients was more likely to be associated with progressive HX in other sites and/or infection. Death from cirrhosis and liver failure per se occurred in one patient 4 years after initial biopsy, but five other children had evidence of cirrhosis on biopsy or at autopsy. The majority of patients with triaditis initially did not have clinical evidence of progressive liver disease although four expired with other manifestations of HX or infection. Conversely, patients showing fibrohistiocytic changes or cirrhosis initially were likely to have continuing or progressive liver disease. Although the liver histology was not diagnostic of HX, the types of portal changes usually predicted the subsequent course of liver disease.

Topics: Biopsy, Needle; Child; Child, Preschool; Cyclophosphamide; Follow-Up Studies; Hepatitis; Hepatomegaly; Histiocytosis, Langerhans-Cell; Humans; Infant; Liver; Liver Cirrhosis; Liver Function Tests; Mercaptopurine; Methotrexate; Multicenter Studies as Topic; Prednisone; Vinblastine

Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone. Ulcerated mucosal lesions may be accompanied by periodontal lesions that present with gingival inflammation, bleeding, recession, necrosis, toothache, dental hypermobility, tooth development abnormalities, and premature tooth loss. This paper presents the case of a five-month-old boy with very early oral manifestations of LCH and describes the combined systemic and dental treatment during a 36-month follow-up. A combined local and systemic approach may be necessary to control oral disease manifestations.

Topics: Cytarabine; Drug Therapy, Combination; Follow-Up Studies; Glass Ionomer Cements; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Infant; Male; Mercaptopurine; Methotrexate; Mouth Diseases; Prednisone; Vinblastine

Topics: Antidiuretic Agents; Antineoplastic Agents; Biopsy; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Mercaptopurine; Pituitary Gland; Prednisolone; Skin; Treatment Outcome; Vinblastine

Topics: Antineoplastic Combined Chemotherapy Protocols; Axilla; Diagnosis, Differential; Groin; Histiocytosis, Langerhans-Cell; Humans; Infant; Male; Mercaptopurine; Prednisolone; Purpura; Scalp; Vinblastine

Topics: Child, Preschool; Glucocorticoids; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Maxillary Sinus; Mercaptopurine; Prednisone; Tomography, X-Ray Computed; Tubulin Modulators; Vinblastine

The study describes an 8-year experience of a single center in managing patients with langerhans cell histiocytosis on the basis of the langerhans cell histiocytosis-III platform. A retrospective case-file review of children diagnosed during 2006 to 2013 was performed. Group 1 (multisystem with risk-organ involvement) patients received an initial treatment of 6 to 12 weeks, followed by continuation treatment to complete 12 months. Drugs included vinblastine, prednisolone, and 6-mercaptopurine. Group 2 (multisystem without risk-organ involvement) patients received a similar treatment, except for 6-mercaptopurine. Group 3 (single-system/multifocal bone disease) patients were treated for a duration of 6 months. Forty-nine patients were treated: 24 (49%), 14 (28.6%), and 11 (22.4%) in groups 1, 2, and 3, respectively. The mean age at diagnosis was 31.6 ± 28.4 months (range, 4 to 120 mo). Five patients abandoned treatment. There were 7 deaths, all in group 1. All patients who died had either a partial response or progressive disease after induction (P=0.000). Among patients with liver involvement, those with sclerosing cholangitis had a greater mortality (P=0.007). A relapse was observed in 12 (24.5%) patients. The frequency of relapse was not different in the 3 groups (P=0.833). The 5-year event-free survival in groups 1, 2, and 3 was 29.3 ± 10%, 58.9 ± 14.6%, and 69.3 ± 15%, respectively (P=0.019). The 5-year overall survival was 100% in groups 2/3 and 68.9 ± 9.8% in group 1 (P=0.011).

Topics: Child; Child, Preschool; Disease-Free Survival; Female; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; India; Infant; Male; Mercaptopurine; Prednisolone; Retrospective Studies; Vinblastine

Topics: Adult; Biomarkers; Biopsy; Colon; Colonic Diseases; Colonoscopy; Drug Therapy, Combination; Female; Gastrointestinal Agents; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Mercaptopurine; Predictive Value of Tests; Prednisolone; Time Factors; Treatment Outcome; Vinblastine

Topics: Antigens, CD1; Antimetabolites, Antineoplastic; Drug Therapy, Combination; Female; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Lacrimal Apparatus Diseases; Mercaptopurine; Methotrexate; Middle Aged; S100 Proteins; Tomography, X-Ray Computed

We report a case of Langerhans cell histiocytosis with a restricted presentation (eosinophilic granuloma of the mandible) complicated by multiple reactivations.

Topics: Antimetabolites; Antineoplastic Agents; Antineoplastic Agents, Phytogenic; Biopsy; Child; Cladribine; Curettage; Eosinophilic Granuloma; Female; Glucocorticoids; Histiocytosis, Langerhans-Cell; Humans; Mandibular Diseases; Mercaptopurine; Methotrexate; Prednisolone; Recurrence; Tomography, X-Ray Computed; Vinblastine

Little information is available regarding effective systemic therapies for adult Langerhans cell histiocytosis (LCH). The Japan LCH Study Group has formulated an ambulatory treatment regimen for adult patients with LCH. In total, 14 patients (median age 43 years, range 20-70 years) with multifocal LCH with biopsy-confirmed histology were enrolled. None had received cytoreductive agents for LCH previously. Four had single system (SS) and ten had multi system (MS) disease. All were treated with the Special C regimen, which consists of vinblastine/prednisolone and methotrexate with daily 6-mercaptopurine for 36 weeks. At the end of the therapeutic regimen, all SS patients achieved no active disease (NAD), and six of the ten MS patients showed a response (NAD in two, partial response in four). At the last follow-up (median 34 months), 11 patients were alive (NAD in eight and active disease in three). Of the three deceased, one died of hemorrhage during the Special C treatment, and two of infections during subsequent therapy. Although this study is limited by the small sample size, this ambulatory regimen shows signs of efficacy for adult LCH. This was particularly evident for patients with multifocal SS disease, but half of those with MS disease also benefited.

Topics: Adolescent; Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Female; Follow-Up Studies; Histiocytosis, Langerhans-Cell; Humans; Male; Mercaptopurine; Methotrexate; Middle Aged; Treatment Outcome; Vinblastine; Young Adult

Topics: Adult; Anus Diseases; Axilla; Combined Modality Therapy; Drug Therapy, Combination; Histiocytosis, Langerhans-Cell; Humans; Lung Diseases; Male; Mercaptopurine; Organ Specificity; Perineum; Prednisolone; Vinblastine

Topics: Biopsy; Bone Diseases; Cyclosporine; Femur; Glucocorticoids; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Infant; Lung Diseases; Male; Mercaptopurine; Methotrexate; Methylprednisolone; Prednisone; Radiography; Skin; Skin Diseases, Papulosquamous

Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.

Topics: Histiocytosis, Langerhans-Cell; Humans; Infant; Liver; Lung; Male; Mercaptopurine; Methotrexate; Prednisone; Vinblastine

Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Cyclosporine; Cytarabine; Female; Histiocytosis, Langerhans-Cell; Humans; Lymphoma, Non-Hodgkin; Mercaptopurine; Methotrexate; Prednisolone; Prednisone; Vinblastine; Vincristine

We report on the thyroid involvement with Langerhans cell histiocytosis (LCH) in a 3-year-old male. The patient presented with goiter and primary hypothyroidism. His goiter caused life-threatening airway obstruction. He developed locally invasive disease 4 years after his response to LCH therapy. LCH should be suspected as a cause of goiter and thyroidectomy is recommended.

Topics: Airway Obstruction; Carcinoma, Papillary; Child, Preschool; Combined Modality Therapy; Goiter; Histiocytosis, Langerhans-Cell; Humans; Hypothyroidism; Male; Mercaptopurine; Methotrexate; Prednisone; Thyroid Diseases; Thyroid Neoplasms; Thyroidectomy; Thyroiditis, Autoimmune

Epstein-Barr virus (EBV) is a herpesvirus for which latent infection in B lymphocytes occurs in most individuals by middle childhood. Clinically significant reactivation of this virus occurs in the context of suppressed cell-mediated immunity, occasionally developing into lymphoproliferative disease (EBV-LPD). EBV reactivation is rarely associated with intensive chemotherapy alone. Here we present the case of a 4-year-old female who developed EBV-LPD as a complication of prolonged immunosuppressive chemotherapy for her multiply-recurrent Langerhans cell histiocytosis (LCH).

Topics: Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Antineoplastic Combined Chemotherapy Protocols; Asparaginase; Cytarabine; Doxorubicin; Drug Therapy, Combination; Epstein-Barr Virus Infections; Fatal Outcome; Female; Herpesvirus 4, Human; Histiocytosis, Langerhans-Cell; Humans; Immunocompromised Host; Infant; Lymphoma, Large B-Cell, Diffuse; Mercaptopurine; Methotrexate; Mycoses; Prednisone; Recurrence; Rituximab; Vinblastine; Vincristine; Virus Activation

In rare cases, patients with Langerhans cell histiocytosis (LCH) develop neurodegenerative CNS disease (ND-CNS-LCH). Management of ND-CNS-LCH has not been established.. We treated five pediatric patients with a combination of intravenous immunoglobulin (IVIG) and chemotherapy (steroid +/- vinblastine +/- 6-mercaptopurine +/- methotrexate). Prior to the therapy, three of the five patients had cerebellar ataxia while the remaining two had abnormal MRI findings without apparent neurological deficits. IVIG was given monthly or twice monthly at the dosage of 250-400 mg/kg/dose.. The four patients administered more than 23 doses of IVIG and chemotherapy remained in a stable condition and did not show significant progression signs in neurological deficits or brain MRI findings during the 30-month follow-up period (median; range: 19+ to 38+) following the initiation of therapy for ND-CNS-LCH.. The IVIG-containing treatment may be promising for ND-CNS-LCH; however, its effectiveness remains to be further tested in more patients as well as in a randomized trial.

Topics: Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Combined Modality Therapy; Histiocytosis, Langerhans-Cell; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Male; Mercaptopurine; Methotrexate; Neurodegenerative Diseases; Vinblastine

The aim of study was to evaluate outcome of international treatment protocol LCH II for children with Langerhans cell histiocytosis treated in FN Motol.. Between November 1995 and December 2003, 46 children were treated, sex ratio M:F 29:17 and median age at diagnosis 6 years 8 months. 28 children (60.9%) suffered from monosystem disease with majority of bone lesions (23 times) with skull predominance (16 times). Surgery was primary treatment modality for monosystem disease. Five children with recurrence were successfully treated by protocol LCH II - LR (3x) and LCH III - LR /G2/, respectively. Eighteen children (39.1%) suffered from multisystem disease. 6 out of 18 patients were treated according to low-risk protocol LCH II - LR and 12 children by high-risk scheme LCH II - HR at the non-randomized branch included etoposide. Recurrence was revealed in 11 patients and 10 of them reached 2nd or 3rd complete remission (CR) by 2 - chlorodeoxyadenosine (CDA) monotherapy, and 1 child reached 2nd CR by LCH II - HR scheme. Two children underwent irradiation after bone lesion excision as well as 1 child as supplemental treatment. Totally, 29 children (63.0%) achieved 1st CR, 14 (30.4%) 2nd CR, 2 (4.4%) 3rd CR, and 1 child died because of LCH progression. There were no severe side effects of chemotherapy. Follow-up median time was 5 years 8 months (range 9 months - 9 years 6 months).. LCH II protocol is safe and effective. Results revealed that treatment of patients with multisystem disease might demand some treatment modification.

Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Drug Therapy, Combination; Etoposide; Female; Histiocytosis, Langerhans-Cell; Humans; Infant; Male; Mercaptopurine; Methotrexate; Prednisone; Remission Induction; Vinblastine

Topics: Adult; Histiocytosis, Langerhans-Cell; Humans; Liver Transplantation; Magnetic Resonance Imaging; Male; Mercaptopurine; Postoperative Complications; Prednisolone; Recurrence; Time Factors; Vinblastine

Langerhans cell histiocytosis (LCH) is an enigmatic disease usually occurring in children. Tumor lysis syndrome (TLS) is a clinical syndrome associated with severe metabolic derangement and oliguric acute renal failure. In this report, we present the clinical course of an infant with advanced LCH who had TLS develop after chemotherapy. Treatment with continuous arteriovenous hemofiltration resulted in effective control of serum uric acid, potassium, creatinine, phosphorus, and blood urea nitrogen levels in the blood.

Topics: Acute Kidney Injury; Allopurinol; Antineoplastic Combined Chemotherapy Protocols; Biomarkers; Blood Transfusion; Combined Modality Therapy; Disease Progression; Etoposide; Fatal Outcome; Female; Fluid Therapy; Hemofiltration; Histiocytosis, Langerhans-Cell; Humans; Infant; L-Lactate Dehydrogenase; Lymph Nodes; Mercaptopurine; Multiple Organ Failure; Neck; Orbital Diseases; Prednisolone; Sodium Bicarbonate; Temporal Bone; Tumor Lysis Syndrome; Uric Acid; Vinblastine; Vincristine

Langerhans cell histiocytosis is an interesting disorder with a variety of presentations and variable outcomes. This study evaluates response to treatment, recurrence, and survival in disseminated Langerhans cell histiocytosis treated at Regional Cancer Centre, Trivandrum, India from 1983 through 1994. Thirty-five patients with disseminated Langerhans cell histiocytosis were seen. Six had group A disease, 21 had group B disease, and eight had group C disease. In group A, five of six patients are disease free at a median follow-up of 48 months. Two had recurrence after initial treatment, which was salvaged. In group B, 13 of 15 patients had complete response after chemotherapy, nine of whom experienced recurrence later. Three of five patients who received irradiation alone experienced recurrence. One died of progressive disease. Two patients were lost to follow-up. Seventeen of 20 are alive with median follow-up of 67 months. In group C, one of eight patients are alive after multiple recurrences. Of the surviving patients, 29% had significant sequelae. In summary, group A and B patients do well with treatment, and most of the recurrences can be salvaged. A significant proportion of patients have sequelae. Newer aggressive protocols must be developed for treating group C patients. Measures to prevent sequelae must also be developed.

Topics: Adolescent; Antineoplastic Agents; Child; Child, Preschool; Female; Histiocytosis, Langerhans-Cell; Humans; Immunosuppressive Agents; Infant; Male; Mercaptopurine; Methotrexate; Prednisolone; Recurrence; Survival Analysis; Treatment Outcome; Vinblastine

Topics: Age Factors; Bone Diseases; Child, Preschool; Drug Therapy, Combination; Female; Histiocytosis, Langerhans-Cell; Humans; Infant; Langerhans Cells; Lung Diseases; Male; Mercaptopurine; Prednisone; Prognosis; Radiotherapy Dosage; Vinblastine

The course of pregnancy is described in a 20-year-old patient who had had active histiocytosis X since infancy. After an acute onset during the first year of life, a disseminated chronic state developed at 18 months of age which persisted until the pregnancy. Long-term therapy with 6-mercaptopurine was instituted at diagnosis and was not discontinued until the 17th week of pregnancy. The course of pregnancy, delivery and puerperium was normal except for elevated LDH, altered protein electrophoresis and anemia. The newborn showed no abnormality.

Topics: Adult; Delivery, Obstetric; Drug Therapy, Combination; Female; Fetus; Histiocytosis, Langerhans-Cell; Humans; Labor, Obstetric; Mercaptopurine; Prednisone; Pregnancy; Pregnancy Complications

Topics: Acute Disease; Child, Preschool; Histiocytosis, Langerhans-Cell; Humans; Infant; Male; Mercaptopurine; Methotrexate; Prednisolone; Vinblastine

Topics: Child; Histiocytosis, Langerhans-Cell; Humans; Mercaptopurine; Prednisone; Recurrence; Remission, Spontaneous; Vinblastine

Topics: Child, Preschool; Chlorambucil; Cyclophosphamide; Histiocytosis, Langerhans-Cell; Humans; Mercaptopurine; Vinblastine

Topics: Child; Histiocytosis, Langerhans-Cell; Humans; Infant; Mercaptopurine

Topics: Child; Genetic Diseases, X-Linked; Histiocytosis, Langerhans-Cell; Humans; Infant; Leukemia, Hairy Cell; Lymphatic Diseases; Mercaptopurine; Prednisone; Radiotherapy; Severe Combined Immunodeficiency; X-Ray Therapy