Primary Congenital and Secondary Acquired Splenic Panhematopenia

Abstract

1. The spleen is an organ of multiple structures and many functions, but in the interests of human health and disease, it is probably far more important pathologically than physiologically. 2. It has been abundantly proved that instability in splenic functional balance toward any one of the essential elements of the blood passing through this organ may be an inherited trait, as in congenital hemolytic icterus. Recognition is now made of a syndrome in which, despite intensive compensatory panmyeloid hyperplasia, indiscriminate elimination of all circulating elements occurs, actually simulating panmyeloid hypoplasia. Splenectomy in such a syndrome is often dramatically curative. "Primary splenic panhematopenia" is suggested as an appropriate descriptive designation. 3. The potentially important role which may be played by the spleen, secondarily involved in a wide variety of syndromes, with the precipitation of varying degrees of peripheral cellular disequilibria, demands careful diagnostic discrimination. A dependable experience in the specific technics by which bone marrow and splenic functions are appraised is essential to sound judgment and clinical acumen. 4. The normal spleen is apparently not essential to life and health at any age and, therefore, may be surgically removed without prejudice to future hemolytopoietic equilibria and longevity. The pathologic spleen may at times constitute a very real hazard to health and an actual threat to survival; in the more acute syndromes, prompt surgical intervention may be lifesaving.

‘Non‐Tropical Idiopathic Splenomegaly’ (‘Primary Hypersplenism’): A Review of Ten Cases and their Relationship to Malignant Lymphomas

“…Even so, a variety of descriptive titles has been used. For instance, Wiseman and Doan (1942) and Heinle and Holden (1949) used the term 'primary splenic neutropenia' and Doan and Wright (1946) 'splenic panhematopenia' for a supposed primary disorder of the spleen in which all blood elements were reduced. Later, othcr cam possibly or probably similar to our own were reported by Dausset, Paraf and Caroli (1952), Hayhoe and Whitby (1955), Gevirtz, Nathan and Berlin (1962) and Weinstein (1964) under the titles 'ankmie hkmolytique hyper-splPnique', 'primary hypersplenic pancytopcnia', 'primary hypersplenism' or 'simple splenic hyperplasia', respectively.…”

Hereditary splenomegaly with hypersplenism

“…The term "hypersplenism" was first introduced by Chauffard (1907) to suggest that the spleen can play a role in the pathogenesis of anemia, leukopenia and thrombocytopenia, or any combination of these manifestations. Decades after this first introduction the term gained popularity when the mechanism, whereby the spleen wrought these alterations, became a matter of controversy (Doan & Wright 1946, Dameshek 1955. The concept of hypersplenism is now well established and denotes the functional state which results in the above mentioned changes, accompanied by a normocellular or hyperplastic bone marrow and, usually, a n enlarged spleen.…”

Splenic Panhaematopenia

“…10, 1947. splenc haemolysis is present, but is due to abnormal properties of the erythrocytes and not to primary haemolytic 'hypersplenism ' (Singer, 1945). On the othr hand Doan et al (1946) hold that formation and destuction of blood elements in the normal spleen are balanced physiologicl functions. This equilibrium may be disturbed by a pathological dysfunction which may affect one of the cellular elements of the blood.…”