Splenic infarction, warm autoimmune hemolytic anemia and antiphospholipid antibodies in a patient with infectious mononucleosis

An 18-year-old woman with infectious mononucleosis developed splenic infarction and severe anemia. Evaluation of her anemia revealed a warm autoantibody and a persistently prolonged partial thromboplastin time that ultimately led to the identification of antiphospholipid antibodies. Although the warm autoantibody disappeared after 2 months, the antiphospholipid antibodies remained persistently positive. To our knowledge, this is the first case report in the literature of this specific constellation of findings, with infectious mononucleosis, warm antibody hemolytic anemia, splenic infarction and persistently positive, high-titer antiphospholipid antibodies occurring in an individual patient.     

Paroxysmal nocturnal hemoglobinuria associated with infectious mononucleosis.

A previously healthy 16-yr-old girl was found to have pancytopenia, low reticulocyte count, a cellular bone marrow, and a negative Coombs test, all coincident with clinical and laboratory evidence of infectious mononucleosis. Symptoms and signs of infectious mononucleosis subsided, but pancytopenia and hemolytic anemia persisted. Sucrose hemolysis and acid hemolysis tests supported a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). After 18 mo, the platelet count is normal, but leukopenia and hemolytic anemia continue. The development of PNH in this patient suggests it may have resulted from an effect of infectious mononucleosis.     

Concomitant infectious mononucleosis and hemolytic icterus

Hemolytic anemia concomitant with infectious mononucleosis, was observed ina 22 year old white male whose history indicates no pre-existing hemolytic disease.Recovery occurred spontaneously.

     

Diagnosis and mechanism of hemolysis in chronic hemolytic anemia with nocturnall hemoglobinuria

1. Studies in vitro and transfusion experiments indicate that the "cause" ofnocturnal hemoglobinuria is an abnormality of the erythrocytes. In vitro, patient’scorpuscles undergo hemolysis in fresh human sera, but only within a pH range of6 to 8, a range more restricted on the alkaline side than the limits within whichisohemolysis will take place.

2. The nonspecific nature of the acid-serum test is emphasized. In addition tonocturnal hemoglobinuria, positive tests for hemolysis may be obtained in thisway with certain "warm" and "cold" hemolysins, and in the presence of markedspherocytosis. The control observations necessary for the diagnosis of nocturnalhemoglobinuria are described.

3. The erythrocytes in nocturnal hemoglobinuria are remarkably sensitive tohemolysis by anti-A (or anti-B) but are not hemolyzed by anti-Rh.

4. It is suggested as a hypothesis that the same abnormality, presumably at thecorpuscular surface, which is the cause of the increased sensitivity to hemolysisby anti-A results in the erythrocytes being fatally sensitive in vivo to a hemolyticfactor distinct from complement and normally present in serum.

     

Infectious mononucleosis and acute hemolytic anemia; report of two cases and review of the literature

1. Two cases of infectious mononucleosis complicated by acute hemolyticanemia are presented. One of these patients had, in addition, an underlyingMediterranean anemia.

2. Hemolytic anemia is a rare complication of infectious mononucleosis.

3. Thirteen similar cases have been collected from the literature, and thecumulative data summarized.

4. Recovery occurred in all cases. Splenectomy was considered necessary inonly two.

5. The etiology of hemolytic anemia in infectious mononucleosis is obscurebut there is good evidence to indicate it may be different in different cases.Hypersplenism initiated by infectious mononucleosis could best explain theauthors’ cases whereas the elaboration of hemolytic antibodies would bestexplain some of the reported cases.

Submitted on October 8, 1954 Accepted on February 14, 1955     

Intracellular protein resembling Russell bodies in malignant lymphomas associated with acquired hemolytic anemia

Large amounts of intracellular periodic acid-Schiff positive protein, closelyresembling Russell bodies tinctorially and histochemically and, to varying degrees, morphologically, were found in the neoplastic cells of three patients withmalignant lymphoma associated with acquired hemolytic anemia. Direct Coombstests were positive in all three of these patients, and in two of them, immuneautoantibodies against red cells were observed in unusually high titers. One ofthe latter also had abnormally high serum globulin levels with cryoglobulinemia.

A similar intracellular protein was present in the neoplastic tissue of seven of acontrol group of 65 unselected cases of malignant lymphoma. The serum globulinwas elevated in four of the seven patients and one of these also had cryoglobulinemia. Definite evidence of hemolytic anemia was noted in one, and suggestive evidence in two of these four patients. A lymph node removed from afifth patient, on whom no serum protein determinations had been made, showedhemosiderosis and marked erythrophagocytosis. This patient had not receivedblood transfusions or other treatment before biopsy was performed.

On the basis of our observations, it is suggested (1) that under certain conditions the neoplastic cells of malignant lymphomas are capable of synthesizingabnormal proteins; (2) that the presence of Russell body-like intracellular PAS-positive material in the neoplastic cells may be the morphologic manifestation ofthis protein synthesis; (3) that these abnormal proteins may play an importantpart in the immunological mechanisms responsible for the development of acquired hemolytic anemia in association with malignant lymphomas.

Submitted on May 20, 1954 Accepted on June 22, 1954     

Intrasplenic microcirculation in rats with acute hemolytic anemia

Intrasplenic microcirculation in rats with acute hemolytic anemia has been studied by means of the microsphere method. Alteration in the intrasplenic microcirculation was observed in the anemic rats. The open circulation was reduced from 98% in the normal rats to 67% in the anemic rats, while the closed circulation was increased from 2% in the normal rats to 33% in the anemic ones. The reduction in the open circulation in the anemic rats appears to be attributed to the congestion in the splenic cords as a result of the retardation of the passage of damaged red blood cells. Restoration of the open circulation in rats on the first day of full recovery from anemia seems to be associated with a decrease in the congestion in the splenic cords.