In the July 1996 issue of the Archives, Quintana et al1 described an adolescent girl with sickle cell anemia and extreme thrombocytosis (platelet count, 2×1012/L). We2,3 have also described thrombocytosis in patients with sickle cell anemia; their platelet counts were higher than those in children who underwent splenectomy, but did not exceed 106/L.
In addition to very high platelet counts, the patients described by Quintana et al showed some leukoerythroblastic features and abundancy of megakaryocytes in the bone marrow, with some erythroid hyperplasia. Although erythroid hyperplasia and erythroblastosis are expected in hemolytic anemia including sickle cell disease, abundance of megakaryocytes and bone marrow picture (Figure 4, right, and Figure 31) make me suspicious about myelofibrosis. Was the patient's bone marrow sample stained for collagen and reticulin?
May I also ask what do the authors mean by monozygous sickle cell anemia? Would it be