thioguanine-anhydrous and Syndrome

A 9-month-old boy with known familial neurofibromatosis type I (NF-1) presented with a clinical and laboratory picture suggestive of juvenile chronic myelomonocytic leukemia (JCMMoL). Chromosomal studies obtained from the bone marrow indicated, however, that he had monosomy 7 syndrome. We believe this is the first reported case of monosomy 7 syndrome in a child with NF in the United States, and that this case complements a recent report of two cases of NF, JCMMoL, and monosomy 7 in Japanese children. Since monosomy 7 syndrome is very difficult to differentiate from JCMMoL or acute nonlymphocytic leukemia (ANLL) unless appropriate chromosomal studies are obtained, we believe it is possible that monosomy 7 may occur with increased frequency in patients with NF-1. Monosomy 7 syndrome might therefore be a significant cause of the known association between NF-1 and nonlymphoid leukemia.

Topics: Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Transplantation; Chromosomes, Human, Pair 7; Combined Modality Therapy; Cytarabine; Daunorubicin; Dexamethasone; Diagnosis, Differential; Etoposide; Humans; Infant; Leukemia, Myelomonocytic, Chronic; Male; Monocytes; Monosomy; Neurofibromatosis 1; Pedigree; Preleukemia; Syndrome; Thioguanine

6-Thioguanine (6-TG), the active metabolite of 6-mercaptopurine and its prodrug azathioprine, are thought to be responsible for clinical efficacy in the treatment of active Crohn's disease. Its use as a therapeutic agent for inflammatory bowel disease (IBD) has been limited to patients who are resistant to or intolerant of other antimetabolites. Short-term experience with this agent has not demonstrated an increased incidence of hematologic or hepatic toxicity; however long-term safety data are scarce. We herein report a patient who developed acute sinusoidal obstruction syndrome after 14 months of successful thioguanine treatment. This is the first report of such a complication in an adult treated with 6-TG for active Crohn's disease.

Topics: Acute Disease; Adult; Antimetabolites, Antineoplastic; Crohn Disease; Hepatic Veno-Occlusive Disease; Humans; Male; Syndrome; Thioguanine

Idiopathic hypereosinophilic syndrome (HES) is a rapidly progressive disease that can result in death within weeks to months of diagnosis in untreated patients. We report two pediatric cases of HES. One patient has remained clinically and hematologically stable for 9 years with vincristine and 6-thioguanine (6TG), and the other has received vincristine alone for 6 months with a good response. The use of long-term vincristine and 6TG or vincristine alone has not been previously reported in the management of HES in children. Vincristine administered every 3-4 weeks with or without 6TG can produce a reduction in the overall white cell count and improvement of symptoms of hypereosinophilia. Although the mortality of patients with HES is high, aggressive treatment can result in significant clinical benefit and improved prognosis in children with this syndrome.

Topics: Child, Preschool; Drug Therapy, Combination; Eosinophilia; Female; Humans; Syndrome; Thioguanine; Time Factors; Vincristine

Topics: Adolescent; Adult; Aged; Child; Child, Preschool; DNA Repair; Drug Resistance; Female; Gene Frequency; Humans; Hypoxanthine Phosphoribosyltransferase; In Vitro Techniques; Infant; Infant, Newborn; Male; Middle Aged; Mutation; Syndrome; T-Lymphocytes; Thioguanine

Three cases of allergic granulomatosis (Churg-Strauss syndrome) are reported. The patients all presented 2-10 years after the onset of pulmonary symptoms with a mononeuritis multiplex, accompanied by sharp pain in the extremities involved. In one patient, the neurological findings improved under treatment with corticoids and cyclophosphamide; the other two patients responded to treatment with thioguanine and cytarabine. In the first patient, the diagnosis was confirmed by a biopsy specimen of the sural nerve.

Topics: Adrenal Cortex Hormones; Adult; Antigen-Antibody Complex; Axons; Cyclophosphamide; Cytarabine; Demyelinating Diseases; Female; Granuloma; Humans; Hypersensitivity; Middle Aged; Muscles; Neuromuscular Diseases; Sural Nerve; Syndrome; Thioguanine; Vasculitis

Topics: Adult; Cytarabine; Eosinophilia; Heart Failure; Humans; Hydroxyurea; Male; Prednisone; Syndrome; Thioguanine

Renal and metabolic complications of tumor lysis during 46 episodes of remission induction chemotherapy were reviewed in 37 patients with American Burkitt's lymphoma. Azotemia occurred in 14 patients, preceding chemotherapy in eight. All of these patients had abdominal tumors. Pretreatment azotemia was associated with elevated lactic dehydrogenase (LDH) and uric acid levels, and sometimes extrinsic ureteral obstruction by tumor. Two patients required dialysis for uric acid nephropathy before chemotherapy was initiated. Following chemotherapy, major complications of tumor lysis (hyperuricemia, hyperkalemia and hyperphosphatemia) were associated with very large tumors, high LDH levels and inadequate urinary output. In patients undergoing diuresis and receiving allopurinol, hyperkalemia or hyperuricemia developed infrequently unless concomitant renal failure ensued. Hyperphosphatemia, which occurred only after chemotherapy, developed in 10 of 32 (31 per cent) nonazotemic and in all azotemic patients. Hemodialysis was required in three post-treatment patients for control of azotemia, hyperuricemia, hyperphosphatemia and/or hyperkalemia. Because of the potential for renal failure caused by precipitation of phosphate, severe hyperphosphatemia is an additional criterion for dialysis in patients with acute tumor lysis syndrome.

Topics: Adolescent; Adult; Antineoplastic Agents; Burkitt Lymphoma; Child; Child, Preschool; Cyclophosphamide; Cytarabine; Drug Therapy, Combination; Humans; Hyperkalemia; Hypocalcemia; Methotrexate; Phosphorus Metabolism Disorders; Prednisone; Syndrome; Thioguanine; Uremia; Uric Acid; Vincristine