Rh deficiency syndrome designates rare individuals who have either absent (Rhnull) or markedly reduced (Rhmod) Rh antigen expression, mild to moderate hemolytic anemia associated with the presence of stomatocytes, and occasional spherocytes on the peripheral blood film. Hemolytic anemia is improved by splenectomy. Here is one example of a case: The hematological aspects of the original case of Rhmod are reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The 51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival. The topic of Rh … Continue reading Rare diseases: Rh deficiency syndrome