Acquired cyclic thrombocytopenia-thrombocytosis with periodic defect of platelet function.
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 x 10(9)/l) were followed regularly by thrombocytosis (700-2300 x 10(9) platelets/l). The period of platelet count fluctuation was about 40 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, thymopentin, high-dose intravenous gamma-globulin and splenectomy were without effect. After 4 years of cyclic platelet and megakaryocyte fluctuations, stable amegakaryocytic thrombocytopenia developed and the patient died of haemorrhagic stroke.
Balduini
C L
CL
Department of Internal Medicine, University of Pavia-IRCCS S. Matteo, Italy.
Stella
C C
CC
Rosti
V
V
Bertolino
G
G
Noris
P
P
Ascari
E
E
D000328:Adult; D001792:Blood Platelets / Q000473:pathology / Q000502:physiology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008533:Megakaryocytes / Q000473:pathology; D010507:Periodicity; D010974:Platelet Aggregation; D010976:Platelet Count; D013921:Thrombocytopenia / Q000503:physiopathology*; D013922:Thrombocytosis / Q000503:physiopathology*