Journal of Hematology & Thromboembolic Diseases
Open Access

Thomas Kuehn

Publications

• Research Article
  Acquired Von Willebrand Syndrome (AVWS) Type 2A as the Presenting Feature of JAK2 Wild type Thrombocythemia in a Child: Effectiveness of Platelet Reduction by Anagrelide
  Author(s): Jan Jacques Michiels, Ulrich. Budde and Thomas Kuehn Jan Jacques Michiels, Ulrich. Budde and Thomas Kuehn
  
  A 9-year-old Caucasian boy presented in 1994 with severe headache, attacks of migraine, aggressive behavior and minor bleeding symptoms. Initial abnormal laboratory data were a platelet count of 1596 × 109/L and slight splenomegaly on echogram. Low-dose aspirin 100 mg/day relieved the cerebral symptoms, but a pronounced spontaneous bleeding tendency became evident. Severe epistaxis, bruises, hematomas and gum bleedings resulted in an iron deficiency state (hemoglobin 5.7 mmol/L,hematocrit 0.30, MCV 77 fl, ferritine 6 ug/l) in November 1995. The combination of mucocutaneous bleeding, high platelet counts (1946 × 109/L), and increase of enlarged megakaryocytes in a bone marrow smear was consistent with the diagnosis of hemorrhagic thrombocythemia. Coagulation studies revealed the presence of an acquired von Willebrand syndrome type 2A featured by a prolonged Ivy bleeding time, near normal .. View More»
  DOI: 10.4172/2329-8790.1000274

  Abstract PDF

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