Observations on the anemia of cryptogenetic splenomegaly. I. Hemolysis

Excessive hemolysis has been shown to occur in patients with cryptogeneticsplenomegaly. This is corrected by splenectomy.

The survival of normal red cells transfused to such patients is greatlyreduced. Following splenectomy normal red cells so transfused survive normally.

The survival of red cells from such patients upon transfusion to healthyrecipients, including a splenectomized recipient, is greatly reduced. Following splenectomy the survival of red cells from such patients upon transfusion to healthy recipients is significantly increased, although still significantly less than normal. However, upon transfusion into splenectomized butotherwise healthy recipients the survival time is normal.

Study of osmotic fragility before splenectomy demonstrates the existenceof a mixed population showing both increased and decreased fragility tohypotonic saline. Following splenectomy there emerges a population withincreased resistance to hypotonic saline.

It is concluded that prior to splenectomy the pathologic spleen exerts aneffect upon circulating erythrocytes including transfused normal red cellswhich results in marked reduction in their survival. Following splenectomya second defect in the erythrocytes becomes apparent and this also resultsin reduction in life-span, although the presence of the spleen would appearnecessary for this reduction to occur. It is suggested that this second defectdevelops as the result of liver damage.

Submitted on February 12, 1957 Accepted on December 25, 1957     

Sarcoidosis manifesting as cardiac sarcoidosis and massive splenomegaly

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. We report an unusual case of sarcoidosis in a woman presenting with cardiac sarcoidosis and massive splenomegaly with a familial history of cardiac sarcoidosis. Cardiac sarcoidosis was diagnosed based on electrocardiogram, echocardiogram, 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG-PET) and skin histological findings. We performed splenectomy to rule out malignant lymphoma, and histological findings confirmed sarcoidosis. After splenectomy, we initiated prednisolone therapy. After 20 months of diagnosis, she was symptom free. Echocardiography and 18F-FDG-PET may be a key diagnostic tool and prednisolone therapy may be safe, effective, and feasible for cardiac sarcoidosis.     

Splenectomy in patients with malignant lymphoma presenting with massive splenomegaly

6 patients with massive splenomegaly but no peripheral adenopathy were diagnosed by splenectomy as having lymphoma; and were found to have widespread disease. Despite the seriousness of their illness at presentation, all have survived for a median time of 101.5 months and reached complete remission.     

Hepatosplenic T cell lymphoma: a unifying entity in a patient with hemolytic anemia,massive splenomegaly,and liver dysfunction

Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL. He initially presented with refractory AIHA, thrombocytopenia, and massive splenomegaly, requiring splenectomy. Histologic examination of his spleen confirmed diagnosis of HSTCL. Approximately 3 months after diagnosis, he was found to have leukemic transformation, representing a secondary malignancy.     

Sarcoidosis manifesting as massive splenomegaly: a rare occurrence

Sarcoidosis is a multisystemic granulomatous disease of unknown origin occurring worldwide and affecting people of all races and ages. This disease manifests most frequently with bilateral hilar lymphadenopathy, pulmonary infiltrates, and skin and ocular lesions. Granulomatous inflammation of the spleen is common in patients with sarcoidosis, but splenic enlargement is unusual and massive splenomegaly quite rare. Splenomegaly is usually homogeneous, but multiple low-attenuating nodular lesions are occasionally seen and easily mistaken for lymphoma, metastases, or infections such as tuberculosis. We describe an unusual case of sarcoidosis in a woman who presented with massive splenomegaly with extensive nodularity that cleared completely with corticosteroid therapy.     

Observations on the anemia of cryptogenetic splenomegaly. II. Expansion of the plasma volume

In cryptogenetic splenomegaly, although there is consistently a significantreduction in hemoglobin concentration and in the red cell count, the totalred cell volume may be reduced, normal or increased.

Following splenectomy the total red cell volume is either within normallimits or is increased, yet significant anemia persists.

Before splenectomy a significant increase in plasma volume is consistentlypresent. Following splenectomy the plasma volume falls, but it remains significantly greater than normal. It would appear that the greater the plasmavolume before splenectomy the greater is the fall following splenectomy.This fall in plasma volume is not a prompt response to splenectomy, for thefall which occurs one month after splenectomy is equalled or exceeded bythat occurring in the succeeding four months.

It is concluded that an expanded plasma volume before splenectomy contributes with the hemolysis previously reported to the development of theanemia encountered in cryptogenetic splenomegaly. Expansion of the plasmavolume at least contributes to the anemia which persists following splenectomy.

Submitted on February 12, 1957 Accepted on December 25, 1957     

Persistent splenomegaly in an adult female with homozygous sickle cell anemia

Sickle cell anemia (SCA) is associated with repeated episodes of erythrostasis in the spleen, which lead to thrombosis and infarction of the spleen resulting in "autosplenectomy" which is usually complete by 8 years of age.

We present a case of a 22-year-old female who presented with complaints of fever, bone pain and joint swelling. On examination she had pallor, icterus and moderate splenomegaly. Her hemoglobin was 7.5 g/dl. Peripheral smear showed many sickled red cells. Slide test for sickling was positive with 2% sodium metabisulphite. Hemoglobin electrophoresis revealed a single band in the hemoglobin S, D, and G region. No band was seen in the HbA & HbA₂ region. HbF level was 0%. USG showed an enlarged spleen with few defined hypoechoeic lesion.

We present this case because of rarity of association of homozygous SCA with splenomegaly in this age group, the confusion that echogenic lesions in spleen can create and to emphasize the risk of sequestration crises, which remains in such cases.     

Persistent splenomegaly in an adult female with homozygous sickle cell anemia

Sickle cell anemia (SCA) is associated with repeated episodes of erythrostasis in the spleen, which lead to thrombosis and infarction of the spleen resulting in "autosplenectomy" which is usually complete by 8 years of age.We present a case of a 22-year-old female who presented with complaints of fever, bone pain and joint swelling. On examination she had pallor, icterus and moderate splenomegaly. Her hemoglobin was 7.5 g/dl. Peripheral smear showed many sickled red cells. Slide test for sickling was positive with 2% sodium metabisulphite. Hemoglobin electrophoresis revealed a single band in the hemoglobin S, D, and G region. No band was seen in the HbA & HbA(2) region. HbF level was 0%. USG showed an enlarged spleen with few defined hypoechoeic lesion.We present this case because of rarity of association of homozygous SCA with splenomegaly in this age group, the confusion that echogenic lesions in spleen can create and to emphasize the risk of sequestration crises, which remains in such cases.