Evaluation of splenomegaly

Diseases

Summary

Splenomegaly generally denotes a palpably enlarged spleen. However, it may also refer to an enlarged spleen detected by an imaging test. It is not uncommon for a radiologist interpreting a chest x-ray to comment that the spleen seems enlarged (usually considered an incidental finding). Splenomegaly can be found in 2.0% to 5.6% of unselected medical outpatients.[1] [2]

An average human spleen is 10 cm long and weighs 150 grams.[3] It may be difficult to palpate an enlarged spleen in the settings of obesity, a muscular abdominal wall, or the inability to sufficiently relax the abdominal musculature. In these cases, spleen size may need to be determined by imaging.

Approach to splenomegaly

It is difficult to create a stepwise algorithmic approach to the patient with splenomegaly.[4] [5] Urgent presentations include sudden pain associated with splenomegaly, splenic sequestration crisis of sickle cell anemia, and splenic vein or portal vein thrombosis. Otherwise, presentation is often incidental on physical exam or imaging. Categorization by presentations such as massive splenomegaly, isolated splenomegaly, or accompanying symptoms could be considered. However, these categories encompass diverse diagnoses. For example, thalassemia, chronic myeloid leukemia, Gaucher disease, hairy cell leukemia, myelofibrosis, and malaria may all present with a markedly enlarged spleen. Isolated splenomegaly is a feature of diagnoses such as splenic marginal lymphoma or benign splenic neoplasms. Accompanying symptoms are nonspecific; for example, fever may occur with lymphomas, malaria, endocarditis, or infectious mononucleosis.

Library

Classic periorbital purpura
Congo red stain blood vessel in a bone marrow biopsy demonstrating green birefringence pathognomonic of amyloidosis
Bone marrow aspirate showing a typical Gaucher cell
ECG of a patient with infective endocarditis. Note the first-degree AV block, nonspecific intraventricular conduction delay, and nonspecific ST-T wave abnormalities
BCR-ABL translocation
Peripheral blood film of a patient with acute myeloid leukemia showing myeloid blasts with an Auer rod
Peripheral blood smear showing leukoerythroblastic reaction: teardrop red blood cells (black arrows), and myelocyte (red arrow) and promyelocyte (blue arrow)
Red cells in sickle cell disease

Citations

Key Articles

Shakoory B, Geerlinks A, Wilejto M, et al. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Ann Rheum Dis. 2023 Oct;82(10):1271-85.[Abstract][Full Text]
Gertz MA. Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management. Am J Hematol. 2023 Feb;98(2):348-58.[Abstract][Full Text]
Kowdley KV, Brown KE, Ahn J, et al. ACG clinical guideline: hereditary hemochromatosis. Am J Gastroenterol. 2019 Aug;114(8):1202-18.[Abstract][Full Text]

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