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Gray platelet syndrome with splenomegaly and signs of extramedullary hematopoiesis: A case report with review of the literature
Author(s) -
Jantunen Esa,
Hänninen Auli,
Naukkarinen Anita,
Vornanen Martine,
Lahtinen Reino
Publication year - 1994
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830460311
Subject(s) - medicine , bleeding diathesis , myelofibrosis , splenectomy , extramedullary hematopoiesis , platelet , blood platelet disorders , haematopoiesis , pathology , myeloproliferative disorders , platelet disorder , bone marrow , surgery , spleen , platelet aggregation , stem cell , biology , genetics
Gray platelet syndrome (GPS) is a rare bleeding disorder characterized by thrombocytopenia, agranular gray platelets in blood films, and almost total absence of platelet alpha‐granules and their constituents. We describe here a rare case of GPS with myelofibrosis and splenomegaly indicating extramedullary hematopoiesis. Splenectomy was followed by normalization of platelet count but the bleeding diathesis continued. Based on a follow‐up of more than 15 years, the slight myelofibrosis in our patient seems to be non‐progressive. We also summarize the major clinical and laboratory features of previously published cases of GPS in order to obtain more comprehensive understanding of this rare disorder. From the clinical point of view, the bleeding tendency in this syndrome generally varies from mild to moderate, and no specific treatment is usually needed. Careful examination of peripheral blood films is necessary for the diagnosis of this rare syndrome. © 1994 Wiley‐Liss, Inc.