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CONGESTIVE SPLENOMEGALY

Chronic venous congestion can cause a form of splenic enlargement referred to as congestive splenomegaly. Venous congestion can be systemic in origin, caused by intrahepatic disorders

that retard portal venous drainage, or may arise from extrahepatic disorders that directly obstruct the portal or splenic veins. All these disorders ultimately lead to portal or splenic vein

hypertension. Systemic, or central, venous congestion is encountered in cardiac decompensation involving the right side of the heart, as can occur in tricuspid or pulmonic valvular disease,

chronic cor pulmonale, or following left-sided heart failure. Systemic passive congestion produces only moderate enlargement of the spleen that rarely exceeds 500 gm in weight.

The only common causes of striking congestive splenomegaly are the various forms of cirrhosis of the liver. The "pipe-stem" hepatic fibrosis of schistosomiasis causes particularly severe

congestive splenomegaly, while the diffuse fibrous scarring of alcoholic cirrhosis and pigment cirrhosis also evokes profound enlargements. Other forms of cirrhosis are less commonly

implicated.

Congestive splenomegaly is also caused by obstruction of the extrahepatic portal vein or splenic vein. This can stem from spontaneous portal vein thrombosis, which is usually associated

with some intrahepatic obstructive disease, or inflammation of the portal vein (pylephlebitis), such as follows intraperitoneal infections. Thrombosis of the splenic vein itself can be

initiated by compression by tumors in neighboring organs, for example, carcinoma of the stomach or pancreas.

Morphology.

Long-standing congestion produces marked enlargement of the spleen (1000 gm or more); the organ is firm and becomes increasingly so the longer the congestion lasts. The weight can

reach 5000 gm. The capsule is usually thickened and fibrous. The cut surface has a meaty appearance and varies from gray-red to deep red, depending on the amount of fibrosis. Often the

white pulp is indistinct. Microscopically, the red pulp is congested in early chronic congestion but becomes increasingly more fibrous

and cellular with time. The increased portal venous pressure causes deposition of collagen in the basement membrane of the sinusoids, which appear dilated owing to the rigidity of their

walls. The resultant slowing of blood flow from the cords to the sinusoids prolongs the exposure of the blood cells to the cordal macrophages, resulting in excessive destruction

(hypersplenism). Foci of recent or old hemorrhage are often present. Organization of these focal hemorrhages gives rise to Gandy-Gamma nodules: foci of fibrosis containing iron and

calcium salts deposited on connective tissue and elastic fibers.

SPLENIC INFARCTS

Splenic infarcts are common lesions. Caused by occlusion of the major splenic artery or any of its branches, in normal-sized spleens they are most often due to emboli that arise from



thrombi in the heart. The spleen, along with kidneys and brain, ranks as one of the most frequent sites within which emboli lodge. The resulting infarcts can be small or large, single or

multiple or can even involve the entire organ. They are usually bland but can be septic when associated with infectious endocarditis of mitral and aortic valves. Infarcts are also common in

markedly enlarged spleens, presumably because the blood supply cannot keep up with the increased demands of the organ.

Morphology.

Bland infarcts are characteristically pale and wedge-shaped, with their bases at the periphery, where the overlying capsule is often covered with fibrin ( Fig. 14-39 ). In septic infarcts, this

appearance is modified by the development of suppurative necrosis. In the course of healing of splenic infarcts, large, depressed scars often develop.

Figure 14-39Splenic infarcts. Multiple well-circumscribed infarcts are present in this spleen, which is massively enlarged (2820 gm; normal: 150 to 200) by extramedullary hematopoiesis

secondary to a chronic myeloproliferative disorder (myelofibrosis). Recent infarcts are hemorrhagic, whereas older, more fibrotic infarcts are a pale yellow-gray color.

Figure 14-40Thymoma. A, Benign thymoma (medullary type). The neoplastic epithelial cells are arranged in a swirling pattern and have bland, oval to elongated nuclei with

inconspicuous nucleoli. Only a few small, reactive lymphoid cells are interspersed. B, Malignant thymoma, type I. The neoplastic epithelial cells are polygonal and have round to oval,

bland nuclei with inconspicuous nucleoli. Numerous small, reactive lymphoid cells are interspersed. The morphologic appearance of this tumor is identical to that of benign thymomas of

the cortical type. In this case, however, the tumor was locally aggressive, invading adjacent lung and pericardium.

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