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July 1, 1961BloodOpen Access

Decreased Erythrocyte Survival in Hemoglobin H Disease As a Result of the Abnormal Properties of Hemoglobin H: The Benefit of Splenectomy

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Authors

DRDemetrios A. RigasOregon Health & Science UniversityRKRobert D. KolerOregon Health & Science UniversityGCGeorge H. CummingsWilford Hall Ambulatory Surgical Center

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Implication

Case series reveals improved erythrocyte lifespan and hemoglobin levels following splenectomy in hemoglobin H disease, highlighting splenic clearance of precipitated mutant hemoglobin.

Key Points

  • To investigate the mechanisms causing shortened erythrocyte survival in hemoglobin H disease and determine the clinical and hematologic benefits of splenectomy.
  • Studied three siblings with hemoglobin H disease, performing splenectomy on two, alongside a relative with hereditary leptocytosis trait.
  • Measured erythrocyte finite lifespan and random destruction rates before and after splenectomy using chromium-51 (Cr51) labeling.
  • Tested in vitro hemoglobin H denaturation and intraerythrocytic inclusion formation using methemoglobin-forming compounds, including amyl nitrite and sulfisoxazole.
  • Erythrocyte finite lifespan lengthened from 40–45 days pre-splenectomy to normal post-splenectomy, accompanied by higher hemoglobin levels, improved exercise tolerance, and reduced random cell destruction.
  • Hemoglobin H irreversibly denatured and formed inclusions at 40–45 days of cell age, driving splenic destruction, while deoxygenation decreased its solubility and triggered random capillary hemolysis.
  • In vitro exposure to amyl nitrite and sulfisoxazole produced intraerythrocytic inclusions in every erythrocyte from affected patients, whereas normal control cells remained unaffected.

Cite This Study

Rigas et al. (1961) studied this question.

synapsesocial.com/papers/6a71320ff44fa9f079defa07https://doi.org/10.1182/blood.v18.1.1.1
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