Michiels J J
Department of Hematology, University Hospital Dijkzigt, Erasmus University Medical School Rotterdam, The Netherlands.
Leuk Lymphoma. 1996 Sep;22 Suppl 1:1-14. doi: 10.3109/10428199609074356.
According to strict morphological, biochemical and cytogenetic criteria Philadelphia chromosome positive essential thrombocythemia and chronic granulocytic leukemia constitute a separate malignant and individual disease entity, whereas Philadelphia chromosome negative essential thrombocythemia, polycythemia vera and agnogenic or megakaryocytic myeloid metaplasia form a chronic proliferation of three hematopoietic cell lines. Histopathology from bone marrow biopsies permits the characterization and diagnostic differention of the various myeloproliferative disorders and appears to be a main and specific diagostic criterion for polycythemia vera and essential thrombocythemia. Hemorrhagic thrombocythemia is a clinical syndrome of recurrent spontaneous mucocutaneous and secondary hemorrhages often preceded by thromboses, extremely high platelet counts, pseudohyperkalemia, increased bone marrow cellularity and frequently splenomegaly. The diagnostic criteria of essential thrombocythemia with paradoxical occurrence of thrombotic events and hemorrhagic manifestations are a platelet count in excess of 1000 x 10(9)/L and increased bone marrow cellularity in the majority of the cases. Erythromelalgia and other microcirculatory ischemic or thrombotic events or accidents in essential thrombocythemia and polycythemia vera already occur at platelet counts in excess of the upper limit of normal. First line treatment options in essential thrombocythemia and polycythemia vera are control of platelet function with low-dose aspirin and reductive control of platelet count and erythrocytes by bloodletting, interferon and busulfan or hydroxyurea monochemotherapy.
根据严格的形态学、生物化学和细胞遗传学标准，费城染色体阳性的原发性血小板增多症和慢性粒细胞白血病构成一种独立的恶性且独特的疾病实体，而费城染色体阴性的原发性血小板增多症、真性红细胞增多症以及特发性或巨核细胞性骨髓化生则形成三种造血细胞系的慢性增殖。骨髓活检的组织病理学有助于对各种骨髓增殖性疾病进行特征描述和诊断区分，似乎是真性红细胞增多症和原发性血小板增多症的主要且特异性诊断标准。出血性血小板增多症是一种临床综合征，表现为反复自发的黏膜皮肤出血和继发性出血，常先有血栓形成，血小板计数极高，假性高钾血症，骨髓细胞增多，且常伴有脾肿大。原发性血小板增多症出现血栓形成事件和出血表现的诊断标准是血小板计数超过1000×10⁹/L，且大多数病例骨髓细胞增多。原发性血小板增多症和真性红细胞增多症中的红斑性肢痛症及其他微循环缺血或血栓形成事件或意外，在血小板计数超过正常上限时就已出现。原发性血小板增多症和真性红细胞增多症的一线治疗选择是用小剂量阿司匹林控制血小板功能，通过放血、干扰素以及白消安或羟基脲单一化疗来减少血小板计数和红细胞数量。
