Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 78-year-old woman was admitted to our hospital because for pancytopenia and splenomegaly. The bone marrow examination revealed hairy cells in 11.6% of all nucleated bone marrow cells. Hairy cells were positive for tartaricresistance acid phosphatase (TRAP) staining, CD2, CD11c, CD19, CD20, sIgD, CD25, CD103 and k chain. In addition, abdominal enhanced computed tomography (CT) demonstrated splenomegaly and multiple nodular early stains in the spleen. She was diagnosed as having typical hairy cell leukemia (HCL). The patient was treated with cladribine infused continuously for 7 days at a dose of 0.09 mg/kg/day, which induced partial remission without severe adverse effects. Purine analogs such as cladribine and pentostatine are considered first-line agents for patients with HCL. Treatment with cladribine achieved a high overall response rate for patients with HCL and was safe even in elderly patients like ours. It is necessary to observe carefully the complications of severe infections, second malignancies and relapse of HCL after treatment with cladribine.
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PMID:[Successful use of cladribine in the treatment of an elderly woman with typical hairy cell leukemia]. 1803 31

Hairy cell leukaemia (HCL) is a rare, clonal, chronic lymphoproliferative disorder commonly seen in males in the middle years of life. Pancytopaenia with moderate to massive splenomegaly is the most common clinical presentation. Diagnosis is made on detecting the lymphocytes with abundant cytoplasm which spread into hair-like processes on peripheral blood and bone marrow smears, thus giving the name, "hairy cell leukaemia". The bone marrow aspirate is frequently a dry tap. The trephine biopsy has the characteristic features of a honey comb appearance and flow cytometry is typically CD103, CD25, FMC7, CD11c, gamma or kappa light chain positive with the classic B lymphocyte markers CD19, CD20, CD79a. Purine analogues followed by granulocyte-colony stimulating factor (G-CSF) to manage the febrile neutropenia is currently the treatment of choice. A 10 year disease free survival is recorded with these management strategies. Experimental use of anti CD20 and CD22 has also shown promising results in the treatment of this disease. We report four cases of HCL diagnosed in a span of two years at the Royal Hospital, Muscat, Oman.
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PMID:Hairy Cell Leukaemia in Oman: Four cases. 2174 80