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Clinical Approach to Isolated Splenomegaly 441 Clinical Approach to Isolated Splenomegaly 441 Clinical Approach to 77 Isolated Splenomegaly MANORANJAN MAHAPATRA, PRAVAS MISHRA, RAJAT KUMAR Patients with splenomegaly may come to medical such as in chronic myeloid metaplasia; 5) infiltrative attention for a variety of reasons. However, patients with such as in sarcoidosis and some neoplasms; and 6) no obvious explanation for an enlarged spleen present neoplastic such as in chronic lymphocytic leukemia and a difficult diagnostic problem. A detailed history taking, the lymphomas. Miscellaneous causes of splenomegaly appropriate clinical examination and relevant include trauma, cysts, hemangiomas, and other investigations is tool for a diagnosis of splenomegaly. malformations. Appropriate investigations in most patients with undiagnosed splenomegaly will yield a diagnosis. CLINICAL SIGNIFICANCE OF SPLENOMEGALY Patients with undiagnosed splenomegaly, who are It might be noted that spleen size is not a reliable otherwise well and who have no evidence of systemic guide to spleen function, because palpable spleens are diseases, particularly if the spleen is minimally enlarged, not always abnormal and hypersplenic spleens are not may be followed with careful and regular observation. always palpable. Patients with emphysema and low diaphragms commonly have palpable but normal-sized NORMAL FUNCTIONS OF SPLEEN spleens. One study showed that 63 (3%) of 2200 healthy In many instances the spleen enlarges as it performs college freshmen had palpable spleens2 and another its normal functions. The four most important normal study showed that almost 5 percent of hospital patients functions of the spleen are 1) clearance of micro- with normal spleens by scan were thought to have organisms and particulate antigens from the blood palpable spleens by their physicians3. In contrast, clinical stream; 2) synthesis of immunoglobulin and properdin splenomegaly rarely noted in immune thrombocyto- factors; 3) destruction of effete or abnormal RBCs; and penic purpura, despite avid destruction of antibody- 4) embryonic hematopoiesis, which can reactivate as coated platelets by the spleen. extra-medullary hematopoiesis in certain diseases. Although palpable splenomegaly can be detected in only a few patients who do not have an obvious MECHANISMS OF SPLENOMEGALY pathophysiologic disorder, the condition should be of Many of the mechanisms of splenic enlargement are interest to the primary care physician because it is exaggerated forms of normal spleen function1. While a generally this physician who detects the abnormality. wide variety of diseases are associated with enlargement The presence of a palpably enlarged spleen must be of the spleen, 6 etiologies of splenomegaly are considered considered a physical finding that demands further primary, including 1) immune response work evaluation. hypertrophy such as in subacute bacterial endocarditis Patients with splenomegaly may come to medical or infectious mononucleosis; 2) RBC destruction work attention for a variety of reasons. Patients may complain hypertrophy such as in hereditary spherocytosis or of left upper quadrant pain or fullness or of early satiety. thalassemia major; 3) congestive such as in splenic vein A splenic infarct, which typically manifests with left thrombosis or portal hypertension; 4) myeloproliferative upper quadrant pain that sometimes radiates to the left 442 Medicine Update shoulder, can be the first clue to the existence of an Table 1: Diagnostic approach to isolated splenomegaly enlarged spleen. Rarely, splenomegaly can initially 1. Does the patient have a known illness that causes spleno- manifest with the catastrophic symptoms of splenic megaly*? rupture. Some patients are found to have splenomegaly • Infections: e.g. malaria, kala-azar, infectious mononucleosis as a result of evaluation for unexplained cytopenias. etc Splenomegaly can be discovered incidentally on • Hematological: e.g. hemolytic anemias, hemoglobinopathis, physical examination. In recent years, splenomegaly has malignancies, myeloproliferative disorders, etc. been frequently discovered on imaging studies of the • Hepatic: e.g. portal hypertension, splenic or portal vein abdomen performed for other purposes. thrombosis Treat and monitor for resolution. CLINICAL EVALUATION OF SPLENOMEGALY 2. Search for an occult disease**. Physical examination is the most practical and cost • Infections: e.g. infective endocarditis effective method of evaluation of splenomegaly4. The • Hematological: e.g. hereditary spherocytosis, polycythemia vera presence of an enlarged spleen can be more precisely determined, if necessary, by liver-spleen radionuclide • Hepatic: e.g. cryptogenic cirrhosis scan, CT, MRI, or ultrasonography. The latter technique • Autoimmune: e.g. systemic lupus erythematosus is the current procedure of choice for routine assessment • Storage disease: e.g. Gaucher’s disease of spleen size (normal = a maximum cephalocaudal • Miscellaneous: e.g. sarcoidosis, amyloidosis, tropical diameter of 13 cm) because it has high sensitivity and splenomegaly, splenic cysts specificity and is safe, noninvasive, quick, mobile, and If found manage appropriately. less costly. CT will frequently give a better view of the 3. Diagnostic splenectomy if consistency of the spleen and can identify splenic tumors • Systemic symptoms are present and suggests malignancy or abscesses that would otherwise be missed. • Focal replacement of the spleen on imaging studies Radionuclide scans such as gallium scans can identify • No other sites are available biopsy active lymphoma or infections. The technetium liver- 4. Monitor closely and repeat studies until the splenomegaly spleen scan can be important in identifying liver disease resolves or a diagnosis is apparent if as the cause of splenomegaly; in patients with • Otherwise well and no evidence of systemic diseases cryptogenic cirrhosis, a technetium liver-spleen scan that • Spleen is minimally enlarged shows higher activity in the spleen than the liver might be the initial hint of liver disease. None of these * Varies with age, associated features and geographical area ** If patient is asymptomatic look for the causes listed in Table 4 techniques is very reliable in the detection of patchy infiltration e.g., Hodgkin’s disease5. Because of the malignancies, congestive splenomegaly and anemias. spleen’s location and its propensity to bleed, needle Splenomegaly is a multi-disciplinary problem. The aspiration or cutting needle biopsy of the spleen is rarely common conditions that result in isolated splenomegaly performed. In general, a splenic “biopsy” involves can be divided into different diagnostic groups i.e. splenectomy, which can be performed at the time of hematological, hepatic, infective, primary splenic laparotomy or with laparoscopy. conditions6. Indian data on splenomegaly are very sparse, however, western literature6 showed the APPROACH TO ISOLATED SPLENOMEGALY prevalence of different diagnostic groups resulting in Splenomegaly may represent a manifestation pri- splenomegaly were hematological: 25-66%, hepatic: mary disease or of associated disease or probably due 12-46%, infective: 13-25%, primary splenic: 2-5% and to the previous illness not recognized4. The approach to others: 8-21%. the patient with undiagnosed splenomegaly must be Proper history taking, appropriate clinical exami- individualized. The list of causes of splenomegaly is nation and relevant investigations are tool for a diagnosis formidable; the possibilities are, however, greatly redu- of splenomegaly. A detailed history (i.e. geographical ced by appropriate clinical evaluation and investigation. area of the residence, duration of splenomegaly, The approach to a patient with an enlarged spleen progressively enlarged splenomegaly, associated signs should focus initially on excluding a systemic illness that and symptoms i.e. fever, jaundice, pain abdomen, joint could explain the splenomegaly (Table 1). A wide variety pain etc, history of alcoholism, family history) is essential of diseases can lead to splenic enlargement; common in for splenomegaly evaluation. The differential diagnosis our country are malaria, leishmaniasis, hematological of splenomegaly differs with the splenic size at presen- Clinical Approach to Isolated Splenomegaly 443 tation in addition to the age of the patient, clinical Table 2: Causes of asymptomatic splenomegaly features, associated hepatomegaly and lymphadeno- 1. Liver disease with portal hypertension pathy. As the prevalence of splenomegaly and relative 2. Splenic vein thrombosis incidence of diseases associated with it are subject to 3. Agnogenic myeloid metaplasia geographical variation, a clinician while evaluating a 4. Gaucher’s disease patient with palpable spleen should keep this factor in 5. Splenic cysts mind. 6. Sarcoidosis In one study, Swaroop, et al6 studied 317 patients 7. Amyloidosis with splenomegaly over a period of 8 years and analyzed 8. Mild hereditary spherocytosis the association of several clinical and laboratory features with different diagnostic groups. Hematological diseases 9. Early stages of polycythemia vera had significant positive associations with massive splenomegaly, lymphadenopathy and blood cytosis i.e. show a focal abnormality, are sometimes indications for erythrocytosis, leucocytosis or thrombocytosis. Hepatic splenectomy8. It is particularly important to avoid diseases had highly significant positive association with splenectomy in a patient with occult liver disease and hepatomegaly, abnormal
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