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)

The usual methods for the functional evaluation of the spleen in man are based on the measurement of a) spleen volume, b) spleen uptake of particles from the circulating blood, and c) morphological abnormalities of circulating erythrocytes. Analysis of clearance curves of damaged autologous erythrocytes, measurement of spleen size or volume by scintillation scanning and counting of erythrocytes with pits by interference contrast microscopy at present are the most widely used techniques. The most frequent causes of acquired functional abnormalities of the spleen are the sickle cell diseases, inflammatory intestinal diseases, immune complex diseases, chronic myelogenous leukemia, irradiation, hemophilia, and the hyposplenism of newborn infants and of the elderly. Usually there is a parallelism enlarged or normal-sized spleen with decreased function, has been observed in sickle cell syndromes and occasionally in chronic myelogenous leukemia and hemophilia.
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PMID:The evaluation of spleen function in man. 267 38

We report a 39-year-old female patient who underwent HLA-identical sibling allogeneic BMT for CML in accelerated phase. Severe pancytopenia refractory to G-CSF associated with progressive splenomegaly and RBC/platelet transfusion dependency were present from day +60 after BMT. MRD assessed by FISH and RT-PCR multiplex for BCR-ABL rearrangement was negative, and complete chimerism was documented by VNTR on days +100, +180, +360 and 2 years after BMT. Splenectomy was performed on day +225 and pancytopenia resolved but chronic extensive graft-versus-host disease developed, with hepatic cholestasis, diffuse scleroderma and sicca-like syndrome. She was sequentially and progressively treated with different immunosuppressive therapy combinations with no clear benefit. On day +940, she presented with infection over the previously present ulcers on both limbs, which culminated in septic shock and death on day +1041. We conclude that, although splenectomy may reverse poor graft function after allogeneic BMT, hyposplenism may trigger or worsen chronic extensive GVHD leading to increased morbidity and mortality.
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PMID:Refractory chronic GVHD emerging after splenectomy in a marrow transplant recipient with accelerated phase CML. 1285 7