Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Retrospective analysis of childhood histiocytoses treated at the Department of Pediatrics, Ramathibodi Hospital from May 1970 till June 30, 1992 to verify the prevalence according to the recent classification, course and prognosis was conducted. Among 120 cases, 54 were Class I or Langerhan Cell Histiocytosis (LCH, previously called Histiocytosis-X). Nineteen cases were Class II: Infection-Associated Hemophagocytic Syndrome (IAHS) and Sinus Histiocytosis. Forty-seven cases were class III which included acute monocytic leukemia, juvenile CML; malignant histiocytosis, HMR, and histiocytic lymphoma. Excellent prognosis (cure) was seen in all cases of Hand-Schuller Christian Disease, eosinophilic granuloma, sinus histiocytosis with massive lymphadenopathy and many cases of Class II (except IAHS). The worse prognosis (100% mortality rate) was seen in HMR and juvenile CML. The intermediate prognosis (50%, 54%, 58.8% and 66.7% mortality rate) was seen in MH, HL, LSD and IAHS respectively. To differentiate between IAHS and MH/HMR, the prominent bone marrow findings in 12 cases of IAHS revealed that every case showed prominent hemophagocytosis by the promono-histiocytes/histiocytes, the maximal total erythroblasts (TE) were only 7.5 per cent except for 3 cases; in which one case with agranulocytosis from co-trimoxazole had 84 per cent erythroid cells, one case with prior co-trimoxazole treatment had 37 per cent TE, the last one had 40 per cent TE with massive GI bleeding while in the recovery stage of DHF. All 5 cases of HMR had prominent hemophagocytosis and increased TE (> 22%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Histiocytoses in children: analysis of 120 cases and the bone marrow findings in infection-induced hemophagocytic syndrome vs malignant histiocytosis. 782 9

We report an interesting case of Malignant Histiocytosis (MH) with polyploid clones characterized by i(17q) and t(9; 22) translocation. A 47-year-old man had lymphadenopthy, splenomegaly and leukopenia at presentation. Bone marrow (BM) cytology showed 10.5% abnormal histiocytes. Karyotypic analyses with R- and G-banding techniques on BM cells revealed complex abnormalities: 88, XXYY, add(2) (p25), -4, -8, -11, i(17q), -21[4]/89, idem, t(9; 22) (q34; q11), +22[26]/46, XY [47], of which, t(9; 22) was confirmed by fluorescence in situ hybridization using a chromosome 22 paint wcp 22+. This patient was treated with interferon-alpha and COP regimen. 10 months later he achieved a complete hematologic and cytogenetic remission (CR). However, relapse occurred one year after achieving CR. At that time, cytogenetic examination showed a new polyploid clone characterized by add(1) (p36), add(2) (p25), -4, -8, t(9; 22), -11, i(17q), -21, +22 in addition to the other two polyploid clones observed previously. RT-PCR indicated that the BCR/ABL transcript (165bp) observed in classic chronic myeloid leukemia, was present, MH with t(9; 22) has not previously been described in the literature. This case may be the first one of MH with t(9; 22), and is likely a secondary event.
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PMID:A Case of Malignant Histiocytosis having Polyploid Clones Characterized by an Isochromosome of the Long Arm of Chromosome 17 [i(17q)] and t(9;22) Translocation. 2740 5