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)

Acquired von Willebrand disease (vWD) has been described in a few patients with chronic myelocytic leukemia (CML). We present here acquired type 2 vWD associated with CML and provide characterization of an inhibitor to von Willebrand factor (vWF) from this patient. His bleeding time was prolonged. Ristocetin-induced platelet agglutination was abolished whereas botrocetin-mediated aggregation was normal. Multimeric analysis of vWF from patient's plasma showed that larger sizes of multimers were reduced. His past and family histories were negative for bleeding tendency. These results suggested that acquired type 2 vWD was present during his clinical course. The inhibitor was purified by Staphylococcal protein A, suggesting an IgG antibody. Both binding of 125I-vWF to GPIb and platelet agglutination by ristocetin were inhibited by the patient IgG with the concentrations of competing substances necessary to inhibit specific binding by 50% (IC50s) of 260 micrograms/ml and 420 micrograms/ml, respectively. However, the IgG had no effect on these studies mediated by botrocetin. The IgG only reacted with intact vWF and a 39/34 kDa fragment of vWF. These results indicate that the recognition of GPIb binding site(s) on vWF by the IgG is a central pathogenesis of acquired type 2 vWD in this case.
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PMID:Acquired type 2A von Willebrand disease in chronic myelocytic leukemia. 887 31

We report a case of a 32-year-old lady with chronic myeloid leukemia (CML) on Imatinib for the past four years and in complete clinical, hematological and molecular remission who presented to us with sudden onset of headache, vomiting and diplopia following self discontinuation of Imatinib for a month. Investigations were suggestive of chronic phase CML (CML-CP) with massive thrombocytosis and magnetic resonance imaging (MRI) of the brain revealed subdural hematoma. Coagulation studies confirmed the diagnosis of Acquired von Willebrand disease (AvWD) 2A because of thrombocytosis. The patient also tested positive for mutation T315I in bcr-abl gene. Treatment of the patient with high dose of Imatinib and hydroxyurea led to normalisation of platelet counts, reversal of coagulation defect and subsidence of symptoms. The present case highlights the importance of diagnosis of AvWD to determine the cause of bleeding in CML and distinguish it from Imatinib-induced bleeding, as prompt treatment with Imatinib can achieve reversal of the condition.
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PMID:A rare case of chronic myeloid leukemia with acquired von Willebrand disease presenting as subdural hematoma. 2688 78