The role of the spleen in congenital spherocytosis

The three-dimensional world of the spleen was explored by scanning electron microscopy on both arterially perfused and nonperfused specimens, as well as on plastic corrosion casts of splenic vasculatures. Of 25 spleens studied, 18 were examples of hypersplenism. These were contrasted to 7 essentially normal spleens taken from children being staged for treatment of Hodgkin's disease whose spleens proved to be uninvolved in the pathologic process. Splenic sinuses in all 25 spleens were typified by a degree of porosity. RBC were caught in the act of entering sinuses from splenic cords. These sinus windows thus represent one end of an "open" circulation pathway. In casts of microvasculature, direct arteriovenous connections were demonstrated, thus establishing an anatomical basis for an often disputed "closed" circulation pathway. Spleens from 7 patients with hereditary spherocytosis had a super abundance of red pulp. Splenic cords were thickened and crowded with spherocytes, many of which presented slightly wrinkled membranes, as were also noted on the peripheral blood RBC. It is possible that these membrane features are unique for HS and reflect the intrinsic membrane abnormality in protein composition. The 7 spleens from chronic idiopathic thrombocytopenic purpura had white pulp as the predominant region. Germinal centers were frequent. Lymphocytes and plasma cells with well-developed microvilli were suggestive that release of antiplatelet antibody might be occurring in white pulp. Platelets were especially notable in peripheral white pulp and marginal zones. Platelet clumps were observed, generally adjacent to spleenic macrophages.

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“…Elective splenectomy is generally effective in controlling the hemolytic anemia and its sequelae (29)(30)(31)(32).…”

Section: The Spleen In Hereditary Spherocytosismentioning

confidence: 99%

The human spleen as revealed by scanning electron microscopy

Barnhart

Lusher

1976

American J Hematol

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“…Studies of splenic pool size in patients with specific diseases such as tropical splenomegaly [10], hereditary spherocytosis [6] and chronic lym phatic leukaemia [3] indicate a relationship between the pool size and the splenic size. In reports of two groups of patients with miscellaneous disor ders associated with splenomegaly a statistical relationship has been es Discussion tablished between pool size and spleen size [4,12].…”

Section: Splenic Pool Size In Myelo-and Lympho-proliferative Syndromesmentioning

confidence: 99%

“…Nevertheless, the enormous volume of pooled blood in pathologically enlarged spleens indicates that a potential reservoir of blood exists which may be mobilised artificially by pharmacological agents [6,10] and it is possible that this may occur under physiological conditions or as a response to haemorrhage. Many surgeons at splenecto my have developed the habit of squeezing the spleen before ligature of the splenic pedicle to give the patient an auto-transfusion.…”

Section: Splenic Pool Size In Myelo-and Lympho-proliferative Syndromesmentioning

confidence: 99%

Factors Influencing Splenic Pooling of Erythrocytes in the Myelo- and Lympho-Proliferative Syndromes

Toghill¹,

Green²

1973

Acta Haematol

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The splenic pool size has been measured in 50 patients with splenomegaly due to myelo- and lympho-proliferative disorders. Splenic pool size was related primarily to spleen size and was not significantly influenced by the pathological disorder. Changes in spleen size due to treatment or progressive disease altered the pool size. The splenic pool could be discharged with adrenaline and noradrenaline.

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Clinical Features and Splenic Pathologic Changes in Patients With Autoimmune Hemolytic Anemia and Congenital Hemolytic Anemia

Chang

Liang

et al. 1993

Mayo Clinic Proceedings

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The role of the spleen in congenital spherocytosis

Cited by 13 publications

References 13 publications

The human spleen as revealed by scanning electron microscopy

The human spleen as revealed by scanning electron microscopy

Factors Influencing Splenic Pooling of Erythrocytes in the Myelo- and Lympho-Proliferative Syndromes

Clinical Features and Splenic Pathologic Changes in Patients With Autoimmune Hemolytic Anemia and Congenital Hemolytic Anemia

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