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Hemoglobin S-C disease with fat embolism

1959; Elsevier BV; Volume: 27; Issue: 4 Linguagem: Inglês

10.1016/0002-9343(59)90048-8

1555-7162

William B. Ober, Michael S. Bruno, Raymond M. Simon, Leo Weiner,

Hematological disorders and diagnostics

1.1. A case of hemoglobin S-C disease in a twenty-eight year old Negro man dying in crisis is presented. 2.2. Postmortem examination revealed extensive intravascular sickling with the formation of conglutinative thrombi in the lungs, liver, kidneys, adrenals and brain. Acute infarction of the bone marrow by sickled erythrocytes was present. Extensive dissemination of embolic fat and marrow elements was found in the lungs, and fat emboli were present in the brain and glomeruli. The spleen was shrunken, showing extensive autoinfarction and siderofibrotic nodules. 3.3. The variability of the clinical picture of S-C disease is dependent upon the proportion of hemoglobin S that is present. The severity of the crisis and the fatal outcome in this patient is explained by the unusually high concentration of hemoglobin S, namely 62 per cent. 4.4. Crisis in this disease is due to intravascular sickling. A possible pathogenetic sequence is suggested, emphasizing the release of fat from infarcted bone marrow, resulting in increased viscosity of the blood, systemic hypoxia as a result of the capillary-alveolar block produced by pulmonary fat embolism, and further intravascular sickling in the lung and other organs as a result of both local deoxygenation and systemic hypoxia. The nature of the initial insult to the bone marrow remains obscure.