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Thrombocytosis and Thrombocythemia These terms refer to an increase of platelets above 800,000/mm3 The condition is generally considered to be a form of myeloproliferative disease In some patients there is an enlarged spleen, polycythemia, chronic myelogenous leukemia, or myelosclerosis In several of our patients no explanation of the thrombocytosis was found They presented with recurrent cerebral and systemic thrombotic episodes, often of minor degree and transient Cytopheresis, to reduce the platelets, and antimitotic drugs (hydroxyurea) to suppress megakaryocyte formation, are helpful in ameliorating the neurologic symptoms In one of the cases we have followed, several small lesions, presumably infarctions, were situated in the white matter and simulated multiple sclerosis Polycythemia Vera This is another myeloproliferative disorder of unknown cause, characterized by a marked increase in RBC mass and in blood volume and often by an increase in WBCs and platelets as well This condition must be distinguished from the many secondary or symptomatic forms of polycythemia (erythrocytosis), in which the platelets and white cells remain normal The slightly increased incidence of thrombosis in primary polycythemia is attributed to the high blood viscosity, engorgement of vessels, and reduced rate of blood ow The majority of patients with cerebrovascular manifestations have TIAs and small strokes, but we have seen one case of sagittal sinus thrombosis With very high hematocrit, sludging of red cells may be seen in the retinal vessels The cause of cerebral hemorrhage in this disease is less clear, although a number of abnormalities of platelet function and of coagulation have been described (see Davies-Jones et al) A wide variety of bleeding disorders such as leukemia, aplastic anemia, thrombocytopenic purpura, and hemophilia may also give rise to cerebral hemorrhage Many rare forms of bleeding disease may be complicated by hemorrhagic manifestations; these have been reviewed by Davies-Jones and colleagues Sickle Cell Disease This inherited disease is related to the presence of the abnormal hemoglobin S in the red corpuscles Clinical abnormalities occur only in patients with sickle cell disease ie, with the homozygous state, and not in those with the sickle cell trait, which represents the heterozygous state The disease, which is practically limited to persons of central African and certain Mediterranean origins, begins early in life and is characterized by crises of infection (particularly pneumococcal meningitis), pain in the limbs and abdomen, chronic leg ulcers, and infarctions of bones and visceral organs Ischemic lesions of the brain, both large and small, are the most common neurologic complications, but cerebral, subarachnoid, and subdural hemorrhage may also occur and the vascular occlusions may be either arterial or venous Disseminated Intravascular Coagulation (DIC) This is perhaps the commonest and most serious disorder of coagulation affecting the nervous system The basic process depends on the release of thromboplastic substances from damaged tissue, resulting in the activation of the coagulation process and the formation of brin, in the course of which clotting factors and platelets are consumed Virtually any mechanism that produces tissue damage can result in the release of tissue thromboplastins into the circulation Thus, DIC complicates a wide variety of clinical conditions overwhelming sepsis, massive trauma, cardiothoracic surgery, heat stroke, burns, incompatible blood transfusions and other immune complex disorders, diabetic ketoacidosis, leukemia, obstetric com-
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plications, cyanotic congenital heart disease, and shock from many causes The essential pathologic change in DIC is the occurrence of widespread brin thrombi in small vessels, resulting in numerous small infarctions of many organs, including the brain Sometimes DIC is manifest by a hemorrhagic diathesis in which petechial hemorrhages are situated around small penetrating vessels In some cases cerebral hemorrhage is quite extensive, similar to a primary hypertensive hemorrhage The main reason for the hemorrhage is the consumption of platelets and various clotting factors that occurs during brin formation; in addition, brin degradation products have anticoagulant properties of their own The diffuse nature of the neurologic damage may suggest a metabolic rather than a vascular disorder of the brain In the absence of a clear metabolic, infective, or neoplastic cause of an encephalopathy, the onset of acute and uctuating focal neurologic abnormalities or a generalized and sometimes terminal neurologic deterioration during the course of a severe illness should arouse suspicion of DIC, and coagulation factors and brin split products should be measured Platelet counts are invariably depressed and there is evidence of consumption of brinogen and other clotting factors, indicated by prolonged prothrombin and partial thromboplastin times In the related illness abbreviated as HELLP mentioned previously, women with eclampsia develop liver failure and thrombocytopenia; the contribution of this limited form of DIC to the eclamptic effects on the nervous system have not been established (see earlier discussion of eclampsia)
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