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Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease

Fernando Peixoto Ferraz de Campos; Cristiane Rúbia Ferreira; Aloísio Felipe-Silva

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Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population.


Anemia, Sickle Cell, Hemoglobin SC Disease, Embolism, Fat, Multiple Organ Failure, Acute Chest Syndrome


Santos Pereira AS, Brener S, Cardoso CS, Proieti AB. Sickle cell disease: quality of life in patients with hemoglobin SS and SC disorders. Rev Bras Hematol Hemoter. 2013;35(5):325-31.

O’Keeffe EK, Rhodes MM, Woodworth A. A patient with a previous diagnosis of hemoglobin S/C disease with an unusually severe disease course. Clin Chem. 2009;55(6):1228-33. PMid: [PMID:19478026]. []

Platt OS, Brambilla DJ, Rosse WFet al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-44. PMid: [PMID:7993409]. []

Huntsman RG, Lehmann H. Treatment of sickle-cell disease. Br J Hematol. 1974;28(4):437-44. []

Zago MA, Falcão RP, Paquini R. Hematologia: fundamentos e práticas. São Paulo: Atheneu; 2004. p. 295-7. Portuguese.

Nagel RL, Fabry ME, Steinberg MH. The paradox of hemoglobin C disease. Blood Rev. 2003;17(3):167-78. []

Powars D, Chan LS, Schroeder WA. The variable expression of sickle cell disease is genetically determined. Semin Hematol. 1990;27(4):360-76. PMid: [PMID:2255920].

Kato GJ, Gladwin MT, Steinberg MH. Desconstructing sickle cell disease: reappraisal of the role of haemolysis in the development of clinical subphenotypes. Blood Rev. 2007;21(1):37-47. PMid: [PMID:17084951] PMCid:PMC [PMID:17084951]. []

Machado RF, Gladwin MT. Pulmonary hypertension in hemolytic disorders: pulmonary vascular disease: the global perspective. Chest. 2010;137(6 Suppl):30S-8S. PMid: [PMID:20522578] PMCid:PMC [PMID:20522578]. []

Villagra J, Shiva S, Hunter LA, Machado RF, Gladwin MT, Kato GJ. Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobinBlood. 2007;110(6):2166-72. PMid: [PMID:17536019] PMCid:PMC [PMID:17536019]. []

Jerath A, Murphy P, Madonik M, Barth D, Granton J, Perrot M. Pulmonary endarterectomy in sickle cell haemoglobin C disease. Eur Respir J. 2011;38(3):735-7. PMid: [PMID:21885420]. []

Savage HO, Ding N, Eso O, Sachdev B, Lefroy DL. Mobile right atrial thrombin a patient with the hemoglobin SC disease. Case Rep Med. 2011;2011:897167.

Amancio TT, Costa ACL, Borsato ML, Marins L, Bacchi LM, Zerbini MCN. Fatal pulmonary thromboembolism associated with hemoglobin SC disease in a 15 year-old boy. Autopsy Case Rep. [Internet]. 2011;1(3):15-22. []

Wade LJ, Stevenson LD. Necrosis of the bone marrow with fat embolism in sickle cell anemia. Am J Pathol. 1941;17(1):47-54. PMid: [PMID:19970543] PMCid:PMC [PMID:19970543].

Tsitsikas DA, Gallinella G, Patel Sneha, Seligman H, Greaves P, Amos RJ. Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non-SS genotypes and a possible association with humen parvovirus B19 infection. Blood Rev. 2014;28(1):23-30. PMid: [PMID:24468004]. []

Dang NC, Jonhson C, Eslami-Farsani M, Haywood LJ. Bone marrow embolism in sickle cell disease: a review. Am J Hematol. 2005;79(1):61-67. PMid: [PMID:15849760]. []

Castro O. Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am. 1996;10(6):1289-303. []

Lehman EP, Moore RM. Fat embolism, including experimental production without trauma. Arch Surg. 1927;14(3):621-62. []

Peltier LF. Fat embolism:a a perspective. Clin Orthop.1988;232:263-70. PMid: [PMID:3289815].

Schuster DP. ARDS: clinical lessons from the oleic acid model of acute lung injury. Am J Resp Crit Care Med. 1994;149(1):245-60. PMid: [PMID:8111590]. []


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