mercaptopurine and Leukemia--Myelomonocytic--Chronic

The triple association of leukemia, xanthogranulomas, and type 1 neurofibromatosis was first described in 1958. Most leukemias were juvenile myelomonocytic leukemias (JMML), usually called juvenile chronic myelogenous leukemia. We describe a 22-month-old female child with neurofibromatosis 1, xanthomagranulomas, and a JMML. Her mother and her brother also had cutaneous café-au-lait spots. Our patient was treated with mercaptopurine and improved. However, 9 months later she experienced a blastic transformation. The presence of xanthomagranulomas and NF1 in a young child should alert to a possible development of JMML, especially in patients with a family history of NF1.

Topics: Antimetabolites, Antineoplastic; Comorbidity; Female; Humans; Infant; Leukemia, Myelomonocytic, Chronic; Mercaptopurine; Neurofibromatosis 1; Xanthogranuloma, Juvenile

We report an interesting case of chronic myelomonocytic leukemia (CMML) with pentasomy 8q resulting from the duplication of isochromosome 8q in a 47-year-old male. His blood picture and myelogram showed CMML and the chromosome study, using R-banding and G-banding techniques, revealed a karyotype of 47,XY,-8,+i(8)(q10)x2. Dual-color fluorescence in situ hybridization (FISH) studies with a #8 centromeric probe and a locus-specific probe for C-myc gene completely confirmed the result of the conventional cytogenetic method. Reverse transcription polymerase reaction (RT-PCR) revealed no BCR/ABL fusion transcript. Hydroxyurea and 6-mercaptopurine therapy did not induce a complete remission and five months later he died of exacerbation of his disease. On reviewing another two cases with pentasomy 8q in the literature, we feel that pentasomy 8q, when present as a sole anomaly, may play a specific role in leukemogenesis and in determining the clinical characteristics such as monocytic involvement and poor prognosis.

Topics: Aneuploidy; Chromosomes, Human, Pair 8; Cytogenetic Analysis; Fatal Outcome; Gene Duplication; Humans; Hydroxyurea; Isochromosomes; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Middle Aged

Myelosuppression is the main hematotoxic effect of 6-mercaptopurine (6-MP), which is an antimetabolite chemotherapy drug. Immune hemolytic anemia associated with this drug has not been previously reported.. A 67-year-old man with chronic myelomonocytic leukemia presented with anemia 2 weeks after 6-MP therapy had been initiated. Additional tests provided laboratory evidence of hemolysis. When treatment was stopped, the patient's condition and laboratory results showed a progressive improvement.. The direct antiglobulin test was positive for IgG. The eluate and the serum were not reactive with panel red cells but reacted with 6-MP-treated red cells, while the normal serum pool was unreactive. The direct antiglobulin test was no longer positive by 20 days after the cessation of 6-MP therapy.. This drug, 6-MP, should be added to the list of drugs that have been reported to cause immune hemolytic anemia by means of the so-called hapten mechanism.

Topics: Aged; Anemia, Hemolytic, Autoimmune; Coombs Test; Humans; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine

The use of peripheral blood as a source of hematopoietic precursors is an alternative to the bone marrow in allogeneic transplantation. Although pediatric allogeneic PBPC experience is limited, there is no reason to believe that the outcome and benefit with PBPC should be different than adults. We describe our initial experience in two children who received PBPC allogeneic transplantation in whom the donors were mobilized with filgrastim. Hematopoietic recovery was achieved on days 14 and 16, and the patients did not develop severe GVHD.

Topics: Adolescent; Bone Marrow; Bone Marrow Cells; Child; Combined Modality Therapy; Female; Filgrastim; Granulocyte Colony-Stimulating Factor; Hematopoietic Stem Cell Transplantation; Humans; Infant; Leukapheresis; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Recombinant Proteins; Remission Induction; Salvage Therapy; Transplantation Conditioning; Transplantation, Homologous

Seventeen patients with myeloproliferative disorders and one patient with chronic myelomonocytic leukemia (CMMoL) were treated with ranimustine++ (MCNU), and the efficacy was evaluated. MCNU was given intravenously by drip infusion at an usual dose of 100 approximately 150 mg with intervals arranged according to the counts of peripheral blood cells. A complete remission was achieved in all 10 patients with chronic myelogenous leukemia (CML) in chronic phase. In three of patients with polycythemia vera (PV) the excellent effects were obtained, and the other 2 cases showed moderate effect. An excellent effect was obtained in both 2 patients with essential thrombocythemia (ET). A patient with CMMoL revealed partial remission. The overall efficacy rate was 100%. The cases with CML needed more long term and much more dose of the drug in order to get remission compared with PV and ET. After remission in both PV and ET, well controlled states were maintained for a relatively long period with no additional administration. In CMMoL, MCNU combined with 6-mercaptopurine also showed remarkable anti-tumor effects. It suggests that MCNU may be one of the useful drugs for the treatment of CMMoL. The side effects observed with MCNU were a slight degree of nausea and vomiting (28%), however they showed no trouble on carrying out the therapy.

Topics: Adult; Aged; Antineoplastic Agents; Drug Administration Schedule; Female; Humans; Infusions, Intravenous; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Middle Aged; Myeloproliferative Disorders; Nitrosourea Compounds; Remission Induction

Topics: Aged; Blood Transfusion; Cyclophosphamide; Humans; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Prednisone; Red-Cell Aplasia, Pure

A patient is presented in whom the diagnoses of chronic myelomonocytic leukaemia (CMML) and erythroblastopenia were simultaneously established. Besides the conventional criteria for both haemopathies, the culture of bone-marrow precursor cells showed lack of growth of the erythroid stem cells. 6-Mercaptopurine given as therapy for CMML failed to induce any favourable changes in erythroblastopenia, which in turn improved with prednisone. Nevertheless, the patients died five months after diagnosis due to acute transformation of the CMML.

Topics: Aged; Anemia; Bone Marrow; Erythroblasts; Humans; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Prednisone

Topics: Adult; Aged; Anemia, Refractory, with Excess of Blasts; Antineoplastic Combined Chemotherapy Protocols; Cytarabine; Daunorubicin; Female; Humans; Leukemia, Myeloid, Acute; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Middle Aged; Myelodysplastic Syndromes; Prednisolone; Remission Induction; Vincristine