Fatal bone marrow embolism in a child with hemoglobin SE disease†
Melissa Rayburg
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Search for more papers by this authorKaren A. Kalinyak
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Search for more papers by this authorAlexander J. Towbin
Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Search for more papers by this authorPeter B. Baker
Department of Pathology, Nationwide Children's Hospital, Columbus, Ohio
Search for more papers by this authorCorresponding Author
Clinton H. Joiner
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Hematology Program, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229Search for more papers by this authorMelissa Rayburg
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Search for more papers by this authorKaren A. Kalinyak
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Search for more papers by this authorAlexander J. Towbin
Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Search for more papers by this authorPeter B. Baker
Department of Pathology, Nationwide Children's Hospital, Columbus, Ohio
Search for more papers by this authorCorresponding Author
Clinton H. Joiner
Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Comprehensive Sickle Cell Center, Cincinnati, Ohio
Hematology Program, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229Search for more papers by this authorA physician or group of physicians considers presentation and evolution of a real clinical case, reacting to clinical information and data (boldface type). This is followed by a discussion/commentary.
References
- 1 Chernoff AI,Minnich V,Nanakorn S, et al. Studies on hemoglobin E.I. The clinical, hematologic, and genetic characteristics of the hemoglobin E syndromes. J Lab Clin Med 1956; 47: 455–489.
- 2 Orkin SH,Kazazian HHJr,Antonarakis SE, et al. Abnormal RNA processing due to the exon mutation of beta E-globin gene. Nature 1982; 300: 768–769.
- 3 Steinberg MH. Compound heterozygous and other sickle hemoglobinopathies. In: MH Steinberg, BG Forget, DR Higgs, RL Nagel, editors. Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management. New York: Cambridge University Press; 2001. pp 786–810.
- 4 Altay C,Niazi GA,Huisman TH. The combination of Hb-S and Hb-E in a black female. Hemoglobin 1976–1977; 1: 100–102.
- 5 Rey KS,Unger CA,Rao SP. Sickle cell-hemoglobin E disease: clinical manifestations and implications. J Pediatr 1991; 119: 949–951.
- 6 Schroeder WA,Powers D,Reynolds RD,Fisher JI. Hb-E in combination with Hb-S and Hb-C in a black family. Hemoglobin 1977; 1: 287–289.
- 7 Masiello D,Heeney MM,Adewoye AH, et al. Hemoglobin SE disease—A concise review. Am J Hematol 2007; 82: 643–649.
- 8 Vichinsky E. Hemoglobin E syndromes. Hematology Am Soc Hematol Educ Program 2007: 79–83.
- 9 Jetsrisuparb A. Development of severe anemia during fever episodes in patients with hemoglobin E trait and hemoglobin H disease combinations. J Ped Hem Onc 2006; 28: 249–253.
- 10 Lukens JN. Abnormal hemoglobins: General principles. In: JP Greer, J Foerster, JN Lukens, et al., editors. Wintrobe's Clinical Hematology, 11th ed. Philadelphia: Lippincott Williams and Wilkins, Philadelphia; 2004. pp 1247–1262.
- 11 Mishra P,Pati HP,Chatterjee T, et al. HbSE disease: A clinico-hematological profile. Ann Hematol 2005; 84: 667–670.
- 12 Knox-Macaulay HH,Ahmed MM,Gravell D, et al. Sickle cell-haemoglobin E (HbSE) compound heterozygosity: A clinical and haematological study. Int J Lab Med 2007; 29: 292–301.
- 13 Lanchant NA. Hemoglobin E: an emerging hemoglobinopathy in the United States. Am J Hematol 1987; 25: 449–462.
- 14 Stocker JT,Dehner LP. Pediatric Pathology, 2nd ed. Philadelphia: Lippincott, Williams and Wilkins; 2002. p 1458.
- 15 Eichhorn RF. Sickle cell-like crisis and bone marrow necrosis associated with parvovirus B19 infection and heterozygosity for hemoglobins S and E. J Intern Med 1999; 245: 103–106.
- 16 Ataga KI,Orringer EP. Bone marrow necrosis in sickle cell disease: A description of three cases and a review of the literature. Am J Med Sci 2000; 320: 342–347.
- 17 Godeau B,Galacteros F,Schaeffer A, et al. Aplastic crisis due to extensive bone marrow necrosis and human parvovirus infection in sickle cell disease. Am J Med 2007; 91: 557–558.
- 18 Rao SP,Desai N,Miller ST. B19 Parvovirus infection and transient aplastic crisis in a child with sickle cell anemia. J Ped Hem Onc 1996; 18: 175–177.
- 19 Dang N,Johnson C,Eslami-Farsani M,Haywood JL. Bone marrow embolism in sickle cell disease. Am J Hematol 2005; 79: 61–67.
- 20 Vichinsky EP,Neumayr LD,Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med 2000; 342: 855–1865.
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