Sickle Cell Anemia (Homozygous S) with Aseptic Necrosis of Femoral Head

Abstract 1. Six patients with sickle cell anemia who exhibit aseptic necrosis of the femoral head are presented. 2. All patients were proven homozygous S by paper electrophoresis. 3. In our material, aseptic necrosis of the femoral capital epiphysis in patients with sickle cell anemia is not an uncommon finding, 12% of 51 cases demonstrating this lesion. 4. The incidence of this lesion may be even greater than we report, inasmuch as some of our patients were asymptomatic, although typical roentgenographic changes of aseptic necrosis of the femoral head were present.

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Aseptic osteonecrosis of the femoral head in patients with sickle cell anemia

Morphologie ◽

10.1016/j.morpho.2020.08.002 ◽

2020 ◽

Author(s):

M. Severyns ◽

L.E. Gayet

Keyword(s):

Femoral Head ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Aseptic Osteonecrosis

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Histopathology of aseptic necrosis of the femoral head in sickle cell disease

International Orthopaedics ◽

10.1007/s00264-010-1121-6 ◽

2010 ◽

Vol 35 (8) ◽

pp. 1145-1150 ◽

Cited By ~ 8

Author(s):

Martin Mukisi-Mukaza ◽

Anne Gomez-Brouchet ◽

Monique Donkerwolcke ◽

Maurice Hinsenkamp ◽

Franz Burny

Keyword(s):

Femoral Head ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease ◽

Aseptic Necrosis

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MANAGEMENT OF AVASCULAR NECROSIS OF FEMORAL HEAD IN SICKLE CELL ANAEMIA BY MAJJA BASTI – A CASE STUDY.

International Journal of Advanced Research ◽

10.21474/ijar01/7034 ◽

2018 ◽

Vol 6 (5) ◽

pp. 313-316

Author(s):

ChandreshwarPrasad Sinha ◽

◽

PradeepKumar Panda ◽

N Parida ◽

AmitKumar harma ◽

...

Keyword(s):

Femoral Head ◽

Avascular Necrosis ◽

Sickle Cell ◽

Sickle Cell Anaemia ◽

Necrosis Of Femoral Head

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Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia

Blood ◽

10.1182/blood.v63.6.1353.1353 ◽

1984 ◽

Vol 63 (6) ◽

pp. 1353-1360 ◽

Cited By ~ 121

Author(s):

MH Steinberg ◽

W Rosenstock ◽

MB Coleman ◽

JG Adams ◽

O Platica ◽

...

Keyword(s):

Gene Mapping ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Hemoglobin Concentration ◽

Reciprocal Relationship ◽

Acute Chest Syndrome ◽

Mean Corpuscular Hemoglobin ◽

Aseptic Necrosis ◽

Alpha Thalassemia ◽

Alpha Globin

Abstract The characteristic clinical heterogeneity of sickle cell anemia (HbSS) may be, in part, a result of its interactions with alpha-thalassemia. Although alpha-thalassemia clearly affects some hematologic features of HbSS, its role in modulating the vasoocclusive severity of disease is not clear. To further explore this relationship, we examined the incidence of painful episodes, acute chest syndrome, aseptic bone necrosis, and leg ulcers in 3 patient groups with sickle cell disease: (1) 2,147 patients over age 2 yr, stratified according to mean corpuscular volume (MCV); (2) 183 patients selected on the basis of microcytosis and elevated HbA2, on whom globin biosynthesis studies were done; and (3) 125 patients who had alpha-globin genotype assigned by restriction endonuclease gene mapping. When patients were stratified by MCV, there was a reciprocal relationship between HbA2 levels and MCV, reflecting the presence of patients with beta o and alpha- thalassemia in the low MCV groups. The erythrocyte indices and HbA2 levels in patients classified as HbSS-alpha-thalassemia, by either globin synthesis studies or gene mapping, were very similar to those previously reported by others. Neither microcytosis, beta o, or alpha- thalassemia appeared to provide any clear protection from the vasoocclusive complication evaluated, and the prevalence of aseptic necrosis was increased in patients with microcytosis over age 20 yr and in groups with alpha-thalassemia. The effects of a reduced MCV and mean corpuscular hemoglobin concentration (MCHC), of possible benefit by themselves, when accompanied by a reduction in hemolysis and rise in hemoglobin concentration, as in HbSS-alpha-thalassemia, may cause sufficient rise in blood viscosity in critical vascular beds to impair blood flow and negate any amelioration of vasoocclusive complications in HbSS.

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Studies on Abnormal Hemoglobins

Blood ◽

10.1182/blood.v9.11.1023.1023 ◽

1954 ◽

Vol 9 (11) ◽

pp. 1023-1031 ◽

Cited By ~ 18

Author(s):

KARL SINGER ◽

A. ZERNE CHAPMAN ◽

SEYMOUR R. GOLDBERG ◽

HERBERT M. RUBINSTEIN ◽

SOL A. ROSENBLUM

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Paper Electrophoresis ◽

Target Cells ◽

Hemoglobin S ◽

Hemoglobin C ◽

Characteristic Features

Abstract Four Negro patients with pure (homozygous) hemoglobin C disease are described, who exhibited the characteristic features of, (1) a hemolytic process with or without anemia, (2) splenomegaly, and, (3) numerous target cells in the film. The method of choice to establish the diagnosis objectively is paper electrophoresis of the stroma-free hemolysate, prepared from the patient’s erythrocytes. No alkali resistant (F) hemoglobin was found in this syndrome (homozygous for hemoglobin C) in contradistinction to sickle cell anemia (homozygous for hemoglobin S) where F hemoglobin is usually present. Splenectomy did not ameliorate the hemolytic process.

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