Clinical and laboratory studies on the marked thrombocythemic states in myeloproliferative disorders. (11 cases)

Abstract

Eleven cases with marked thrombocytosis over 100×104/cmm were encoutered in the past 10 years. All of them belonged to myeloproliferative disorders, including 6 cases with thrombocythemia hemorrhagica, 3 cases with chronic myelocytic leukemia, each one case of panmyelosis in leukemic stage and of primary myelofibrosis in leukemic stage.In 4 out of 6 cases with thrombocythemia hemorrhagica, definite clinical hemorrhagic tendency were observed such as gingival, postoperative, gastrointestinal bleeding. In 2 cases, intramuscular hematoma just as a simillar from to classic hemophiliacs were occured. These symptomes were seemed to relate clearly with some of the abnormal laboratory data. That is, prolongation of bleeding time (PTT), plasma clot time (PCT) and platelet function defects were revealed in all 4 cases. Low platelet retention rate and low aggregation rate by ADP and collagen were seen. Treatment with Busulfan (3 cases), Busulfan+P32 (1 case) were done without any side effects nor leukemic transformation.Platelet disfunctions seemed to be sustained continually even when platelet count decreased after treatment, although clinical hemorrhagic episodes were disappeared with normalization of bleeding time, PTT and PCT. Afterall Busulfan therapy was thought to be effective to control the hemorrhagic diathesis. In other two cases with thrombocythemia hemorrhagica there was no hemorrhagic diathesis.In 3 cases with CML, no obvious clinical bleeding evidences were seen. On laboratory data, there were no abnormal prolongation of PTT nor bleeding time. Decreased platelet functions were seen in one case which had had bleeding tendency one 13 years ago when she was diagnosed to be CML for the first time.Etiological consideration of thrombocytosis and/or thrombocythemia in myeloproliferative disorders was discussed.