English Journal

A congenital haemolytic anaemia with thermal sensitivity of the erythrocyte membrane.

Zarkowsky HS, Mohandas N, Speaker CB, Shohet SB.AbstractMicrospherocytes, measuring 2-3 mum in diameter, and cells with blunted projections or triangular in shape characterized the erythrocoyte morphology in three children with congenital haemolytic anaemia. Since the erythrocyte morphology resembled that associated with thermal injury, heat-induced changes in erythrocyte morphology and membrane composition were studied. Erythrocytes developed filaments and spheroid bodies which fragmented, resulting in microspherocyte transformation. Normal cells required exposure to 49 degrees C, whereas the patients' cells fragmented at 45 degrees C. Fragmentation was also observed during incubation of patients' cells at 37 degrees C for 17h. The heat-induced transformation of the patients' cells was associated with an increase in the membrane cholesterol:phospholipid and cholesterol:protein ratios. The phospholipid:protein ratio was unchanged. This suggests that fragmentation produces a selective loss of membrane components. Splenectomy ameliorated the haemolytic process. We propose that the patients' red-cell morphology is the result of in vivo fragmentation, and that the spleen is the major site of microspherocyte and poikilocyte destruction.
British journal of haematology.Br J Haematol.1975 Apr;29(4):537-43.
Microspherocytes, measuring 2-3 mum in diameter, and cells with blunted projections or triangular in shape characterized the erythrocoyte morphology in three children with congenital haemolytic anaemia. Since the erythrocyte morphology resembled that associated with thermal injury, heat-induced chan
PMID 1191563

Molecular mechanism of hemolytic anemia in homozygous hemoglobin C disease. Electron microscopic study by the freeze-etching technique.

Lessin LS, Jensen WN, Ponder E.AbstractErythrocytes from a patient with homozygous hemoglobin C disease were subjected to gradual osmotic dehydration by incubation in hypertonic saline. Serial observations of these cells before and after 4 and 12 hr incubation were carried out by means of interference, Soret absorption, polarization microscopy, and the electron microscope employing the freeze-etching technique. Light microscopic studies showed a progressive contraction of cellular contents into central masses which, after 12 hr dehydration, formed birefringent intracellular hemoglobin crystals in 50-75% of the cells. Electron microscopic study of freeze-etched replicas of these cells at 0, 4, and 12 hr of dehydration reveals progressive aggregation, alignment, and crystallization of hemoglobin molecules. Molecular aggregation found in C-C cells prior to osmotic dehydration was not seen in normal erythrocytes. Aggregation and packing varied from cell to cell. Reticulocytes showed a loosely packed aggregate mesh-work; older cells showed variation of molecular packing, which appeared tightest in cells corresponding to microspherocytes. With further loss of intracellular water, aggregates coalesced into patterns of tighter molecular packing with small regions of alignment, and, finally, crystallization occurred. Hemoglobin molecules measuring 70 A in diameter were readily identified within the period patterns of intracellular crystals. These findings suggest that the hemoglobin C molecules within C-C erythrocytes exist in an aggregated state. As the cell ages, intracellular water is lost and intermolecular distance decreases, hemoglobin C molecules polymerize into intracellular crystals. This pathological behavior of hemoglobin C is associated with a charge alteration conferred by the substitution of beta-6-lysine for glutamic acid on the external surface in the A-helix region of the beta-chain of the molecule, possibly increasing intermolecular attraction. Molecular aggregation accounts for the increased rigidity of C-C cells which leads to accelerated membrane and water loss with resultant microspherocyte formation. The microspherocyte, with highest intracellular hemoglobin concentration, rapidly undergoes intracellular crystallization, and is sequestered and destroyed by reticuloendothelial elements.
The Journal of experimental medicine.J Exp Med.1969 Sep 1;130(3):443-66.
Erythrocytes from a patient with homozygous hemoglobin C disease were subjected to gradual osmotic dehydration by incubation in hypertonic saline. Serial observations of these cells before and after 4 and 12 hr incubation were carried out by means of interference, Soret absorption, polarization micr
PMID 5807277

Japanese Journal

Hereditary spherocytic elliptocytosisの一家系

櫻木志津,三谷紀之,田中芳紀,松井久末子,松田万幸,篠原健次,大久保正士
山口医学 53(4・5), 231-235, 2004-10-31
… 系を報告する.発端者の娘には溶血性貧血を,父には溶血を認めたが,貧血はなかった.当初は遺伝性球状赤血球症hereditary spherocytosis(HS)と考えられていたが,発端者の赤血球の大部分は楕円赤血球ovalocyteでありmicrospherocyteも少数認められた.赤血球膜蛋白の分析では発端者ではspectrin,父ではprotein 4.1の量の減少を認めた.今回の結果のみでは本症の病態および親子の病型の違いについては十分には説明できず将来的には遺伝子異 …
NAID 110002774433

遺伝性球状赤血球症の2家系:--走査電顕像を中心として--

星野明彦
順天堂医学 30(1), 59-66, 1984
… また球状赤血球は必ずしもmicrospherocyteではなく, 大小さまざまなものが混在していた. …
NAID 130004716106

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