Premium
Acquired cyclic thrombocytopenia‐thrombocytosis with periodic defect of platelet function
Author(s) -
Balduini Carlo L.,
Stella Carmelo Carlo,
Rosti Vittorio,
Bertolino Giampiera,
Noris Patrizia,
Ascari Edoardo
Publication year - 1993
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1993.tb03214.x
Subject(s) - platelet , thrombocytosis , thymopentin , megakaryocyte , medicine , splenectomy , bone marrow , clot retraction , megakaryocytopoiesis , endocrinology , gastroenterology , haematopoiesis , biology , platelet aggregation , spleen , stem cell , genetics
Summary A periodic fall of platelet number characterizes an acquired pathological condition named cyclic thrombocytopenia. We observed a patient in whom the episodes of thrombocytopenia (platelet number less than 50×10 9 /l) were followed regularly by thrombocytosis (700–2 300 × 10 9 platelets/l). The period of platelet count fluctuation was about 30 d. Morphological examination of bone marrow showed the cyclic disappearance of mature and immature megakaryocytes: bone marrow cultures revealed a periodic severe defect of both multilineage and single‐lineage progenitor cell growth. When platelet count was falling, a mild defect of platelet aggregation and ATP release was observed. while platelet function was normal when platelet count was rising. Prednisone. thymopentine. high‐dose intravenous γ‐globulin and splenectomy were without effect. After 4 years of cyclic platelet and megakaryocyte fluctuations, stable amegakaryo‐cytic thrombocypenia developed and the patient died of haemorrhagic stroke.