Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic neutrophilic leukemia (CNL) is a rare hematologic disorder usually presenting with a persistent neutrophilia in the leukemoid range (WBC > 40-50 x 10(9)/1) and consisting largely of mature neutrophils. Patients have no obvious cause for an elevated white count and typically have an elevated leukocyte alkaline phosphatase score, hepatosplenomegaly, elevated vitamin B12 and are Philadelphia chromosome-negative. CNL has occasionally been associated with paraproteinemia or outright myeloma. Dysplastic features within the neutrophils in CNL have rarely been reported. We report the clinical, pathological and cytogenetic features of a case of CNL in an elderly white female initially diagnosed with refractory anemia with excess blasts, which subsequently progressed to CNL.
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PMID:Chronic neutrophilic leukemia evolving from a myelodysplastic syndrome. 935 49

A 77-year-old man was referred to our hospital because of elevated LDH and leukoblastosis in the peripheral blood in June 2002. Physical examination revealed neither hepatosplenomegaly nor superficial lymphadenopathy. A bone marrow film showed dysmegakaryocytopoiesis with many micromegakaryocytes and MPO-positive blasts appearing in 20-30% of NCC. A diagnosis of MDS (RAEB-t) was made. Blastic cells were positive for CD13, 33, 34 and HLA-DR. Karyotypic analysis at diagnosis revealed 46XY, inv(3) (q21q26), t(9;22) (q34; q11) and minor-BCR/ABL chimeric m-RNA was detected by RT-PCR. Mild chemotherapy (low dose Ara-C etc) was given but the disease progressed to the AML stage with thrombocytosis in August. In September imatinib was given because of Ph positivity, but the effect was transient. In October massive leukocytosis with myeloblastosis was uncontrollable. In December 2002 the patient died of pneumonia, after a total course of 7.5 months. This rare case with Ph chromosome and 3q21q26 syndrome showed a poor prognosis as previously reported.
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PMID:[3q21q26 syndrome with minor-BCR/ABL type Ph chromosome]. 1497 33