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)

Pure red cell aplasia (PRCA) was found in a male patient with chronic myelocytic leukemia after major ABO incompatible bone marrow transplantation (BMT). He had blood group O, and received BMT from an HLA identical sibling (blood group A). Erythrocyte-depleted marrow was transplanted. Methotrexate for short time and cyclosporine (CyA) were used for graft versus host disease (GVHD) prophylaxis. Engraftment of neutrophils and platelets were observed on day 14 and 22, respectively. The Ph1 chromosome disappeared on day 133. However engraftment of erythrocytes was not observed on day + 280. Bone marrow puncture revealed depletion of erythrocyte precursors. Anti-A isoagglutinin was persisted. There was no evidence of acute or chronic GVHD. Administration of prednisolone, discontinuance of CyA and subcutaneous infusion of recombinant human erythropoietin failed to improve PRCA. Bolus methylprednisolone (m-PSL) therapy started on day 284 resulted in rapid increase in reticulocyte counts within 6 days, which was followed by normal hemoglobin concentrations. We conclude that bolus m-PSL may be one treatment for PRCA after BMT.
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PMID:[Treatment with bolus methylprednisolone for pure red cell aplasia after ABO incompatible bone marrow transplantation in a patient with chronic myelocytic leukemia]. 869 68

Pure red cell aplasia (PRCA) is a rare disorder which is associated with thymoma, viral infections and autoimmune diseases. A few cases of PRCA during the clinical course of CML have been reported and these usually terminate in blastic crisis and death, suggesting a poor prognosis. However, only one case of Philedelphia chromosome negative, Bcr-Abl positive CML associated with PRCA has been reported. Here, we present a second case report of a Philedelphia negative, Bcr-Abl positive CML associated with PRCA who was unresponsive to all the chemotherapeutic regimens. We conclude that the present case supports the idea that the development of PRCA in the course of CML may be a bad prognostic sign.
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PMID:Philadelphia negative, Bcr-Abl positive chronic myeloid leukemia associated with pure red cell aplasia. 1286 88

The patient was a 47-year-old man who was diagnosed in 1989 as having chronic myelogenous leukemia (CML). He had been treated with interferon-alpha (IFN-alpha) and hydroxyurea. In August 1999, he was admitted to our hospital for examination of severe anemia and increased platelet count. On admission, his hemoglobin level was 6.3 g/dl, reticulocyte count was 0.7%, WBC count was 5,100/microliter, and platelet count was 57.3 x 10(4)/microliter. Bone marrow aspiration showed myeloid hyperplasia and near absence of erythroblasts. Bone marrow karyotype analysis showed a Ph chromosome with additional abnormalities. Pure red cell aplasia (PRCA) with accelerated-phase CML was considered. The IFN-alpha therapy was discontinued. Hydroxyurea at an increased dosage was effective in controlling the CML. In contrast, administration of cyclosporin A was not effective for the PRCA. The patient's condition was later complicated by acute hepatitis C virus infection. The IFN-alpha was restarted to control the CML and hepatitis. The patient remained erythroblastopenic and transfusion-dependent for more than 2 years. Association of CML and PRCA is rare. We discuss the mechanisms underlying PRCA occurring during the course of CML.
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PMID:[Pure red cell aplasia occurring during the course of chronic myelogenous leukemia]. 1499 37

Tyrosine-kinase inhibitors (TKIs) represent the only hopes for long-term survival for patients with chronic myeloid leukaemia (CML) and gastrointestinal stromal tumours. Thus, uninterrupted use of TKIs is of importance in such patients. Pure red cell aplasia (PRCA) is a rare disorder, not previously known to be associated with TKIs. We present, to the best of our knowledge, the first case of a patient with CML who developed PRCA secondary to both imatinib and nilotinib. Although PRCA was controlled on withdrawal of TKI, TKI continuation in the patient with CML is important. So we treated him with prednisone, but his haemoglobin started to drop on resumption of imatinib. He was changed to nilotinib but again developed PRCA, which did not improve with steroids. We treated him with cyclosporine and were able to reintroduce nilotinib at a reduced dose without further complications. This case report makes physicians aware of this rare complication of TKIs and also provides encouragement that PRCA could be controlled and TKI continued.
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PMID:TKI-induced pure red cell aplasia: first case report of pure red cell aplasia with both imatinib and nilotinib. 2784 12