Hospital Medicine

Splenomegaly

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Splenomegaly

I. Problem/Condition.

Splenomegaly is the enlargement of the spleen. It is a finding that represents a broad range of conditions. It may be associated with hypersplenism, which describes an overactive functional state of the spleen causing potentially life-threatening cytopenias. Splenomegaly, even in the absence of functional hypersplenism, is an important finding that warrants additional and careful work-up.

Clinicians encounter splenomegaly in three specific circumstances. First, splenomegaly is a common incidental finding on abdominal imaging. Second, splenomegaly may be also be found incidentally on routine physical examination of the abdomen. Third, clinicians often seek out the exam finding of splenomegaly when there is an increased suspicion for an underlying disease process which may present with splenomegaly.

Splenomegaly by definition is based on splenic weight. Obviously, it would require either splenectomy for the patient or autopsy examination for a clinician to come to this conclusive definition for splenomegaly. The finding of splenomegaly on physical examination by palpation is an important one that should not be ignored, but the clinician should realize that this finding may not represent true splenomegaly or clinically significant disease.

The spleen is located in the abdomen and lies against the diaphragm at the level of the 9th, 10th, and 11th ribs on the left side of the body just posterior to the midaxillary line. When enlarged, the spleen expands downward below the costal margin and actually moves more anterior and medial into the abdomen. Both body habitus and degree of splenic enlargement will affect the sensitivity of palpating the spleen on physical exam. It should be noted that the tip of the spleen may be palpable in a small percentage of normal adults.

Typically, confirmation of splenomegaly on physical examination is achieved through radiologic evaluation. Many definitions for splenomegaly in radiology exist depending upon the imaging modality as well as the technique used by the radiologist for calculating splenic size. There is no single definition that has been adopted or agreed upon and thus confirmation of splenomegaly through radiologic interpretation may vary based on a radiologist’s subjectivity and indices used to define splenomegaly.

II. Diagnostic Approach

A. What is the differential diagnosis for this problem?

Splenomegaly is almost always due to an underlying secondary medical condition. The differential for splenomegaly is quite extensive, representing potentially serious as well as benign diseases. The many causes of splenomegaly can be grouped in common categories of disease: hematologic, infectious, congestive, inflammatory, neoplastic, infiltrative, and other miscellaneous causes of splenic enlargement such as benign cysts or hematoma(s) which may occur with trauma.

Hematologic, infectious and congestive (due to liver disease) categories account for the majority of disorders causing splenomegaly in adults.

Hematologic causes of splenomegaly can be further divided into additional categories: lymphomas, leukemias, myeloproliferative diseases, congenital and acquired causes of hemolysis, or increased red blood cell turnover. Both non-Hodgkin and Hodgkin lymphomas are associated with splenomegaly. Both acute and chronic leukemias as well as the subtype hairy cell leukemia are associated with splenomegaly.

Myeloproliferative diseases associated with splenomegaly include chronic myelogenous leukemia, primary myelofibrosis, polycythemia vera, and essential thrombocytosis. Congenital conditions associated with hemolysis and increased red blood cell turnover that cause splenomegaly include: hereditary spherocytosis and hemoglobinopathies, especially alpha and beta thalassemias and sickle cell-hemoglobin C (or hemoglobin SC disease). Acquired conditions associated with hemolysis and increased red blood cell turnover that cause splenomegaly include: autoimmune hemolysis, megaloblastic anemia, and paroxysmal nocturnal hemoglobinuria.

Infectious causes of splenomegaly can be further divided into 3 categories: acute, subacute/chronic, and tropical/parasitic infections. Acute infectious causes of splenomegaly include: infectious mononucleosis, viral hepatitides, cytomegalovirus, sepsis, splenic abscess(es), typhoid, and toxoplasmosis. Subacute/chronic infectious causes of splenomegaly include: subacute bacterial endocarditis, tuberculosis, brucellosis, syphilis, and human immunodeficiency virus (HIV). Tropical/parasitic infectious causes of splenomegaly include: malaria, leishmaniasis, and schistosomiasis.

Congestive causes of splenomegaly include: liver cirrhosis (most common), thrombosis or obstruction of the portal, hepatic, or splenic veins, and heart failure.

Inflammatory causes of splenomegaly include: collagen vascular diseases, especially systemic lupus erythematosus (SLE) and rheumatoid arthritis, and granulomatous diseases, especially sarcoidosis.

Neoplastic causes of splenomegaly include: benign hemangiomas or malignant lesions such as metastases from some primary cancer.

Infiltrative causes of splenomegaly include: amyloidosis (primary or secondary) and Gaucher’s disease.

Miscellaneous causes of splenomegaly include: cysts and hematomas.

B. Describe a diagnostic approach/method to the patient with this problem

Clinicians should take a systematic but also targeted approach in evaluating patients with splenomegaly. A thorough history and physical examination is crucial in order to narrow the broad differential for this finding. The history, including addressing portions of the review of systems, family history, and social history, should focus on symptoms and risk factors for the above differential diagnoses.

Likewise, the physical examination, beyond evaluating for splenic enlargement, is important in determining if characteristic signs are present for certain disorders such as heart failure, endocarditis, liver disease, acute/subacute/chronic infections, hematologic/oncologic disorders, and inflammatory conditions. Clinical information gained from the history and physical will help determine what additional laboratory or diagnostic testing needs to occur.

In general, most patients should have fairly comprehensive laboratory testing performed from the beginning of the evaluation once splenomegaly is confirmed with either ultrasonography or computed tomography. Following the initial evaluation, clinicians should review all information gained with the patient and then decide what additional history, physical exam, and diagnostic testing may be needed to help diagnose or clarify a diagnosis for the underlying cause of splenomegaly. Expert subspecialty consultation may be needed for additional work-up or management as indicated.

1. Historical information important in the diagnosis of this problem.

Questions related to splenomegaly:

  • Do you have any abdominal pain, discomfort or fullness?

  • Have you noticed any growths or masses in your abdominal region?

  • How is your appetite and have you noticed any early satiety when eating?

Questions related to possible underlying conditions:

  • Have you had any fevers, chills or night sweats? If so, how long have you had these symptoms and have you noted any temporal patterns to the symptoms?

  • Have you had any unplanned weight loss? If so, how much weight have you lost and over what time interval?

  • Do you feel fatigued?

  • Have you noticed any shortness of breath or decreased exercise capacity?

  • Do you have a cough? If so, is it productive or how long have you had this symptoms? Have you ever coughed up blood or noted blood in your sputum?

  • Do you have a sore throat?

  • Have you noticed any changes in your vision or noted any eye pain?

  • Have you noticed any yellowing of your skin color or eyes?

  • Have you noticed any skin pallor?

  • Are you prone to easy bruising or bleeding?

  • Have you or do you feel you suffer from frequent colds or infections?

  • Have you noticed any swollen or painful lymph nodes?

  • Do you have any trouble lying flat or do you sleep on multiple pillows at night?

  • Have you ever awoken at night due to shortness of breath?

  • Do you have any leg swelling?

  • Have you noticed any skin rashes or nodules?

  • Have you noticed any joint pain or swelling?

Questions related to past medical/surgical history:

  • Do you have a personal history of any type of cancer?

  • Do you have chronic infections such as HIV or viral hepatitis? If no, have you ever been tested for these conditions?

  • Do you have any history of heart failure or heart disease?

  • Have you ever been told you have a heart murmur?

  • Have you had any recent medical procedures including dental or surgical procedures?

  • Do you have any history of cirrhosis or liver disease?

Questions related to family history:

  • Do you have a family history of any type of cancer?

  • Do you have a family history of any congenital or inherited disorders?

  • Do you have a family history of any blood disorders?

Questions related to social history:

  • Do you drink alcohol? If so, how much and how often?

  • Do you use illicit drugs? If so, have your ever used intravenous or injection drugs?

  • Have you had any recent travel?

  • Have you ever traveled abroad? If so, when and where did you travel? During your travel abroad, did you ever swim in any fresh or stagnant bodies of water?

  • Have you had any exposure to pets?

  • Have you had any exposure to wild animals or mosquitoes?

  • Have you had any recent sick contacts?

  • Have you ever been incarcerated?

  • Have you ever been homeless?

  • Can you describe your personal sexual history and contacts?

2. Physical Examination maneuvers that are likely to be useful in diagnosing the cause of this problem.

  • Palpation of the spleen (use percussion to localize if having difficulty finding the organ)

  • Palpation of cervical and groin lymphadenopathy

  • Eye/tongue/tympanic membrane/skin examination for jaundice

  • Conjunctival and skin examination for pallor

  • Oropharynx examination for pharyngitis or exudate

  • Evaluation for stigmata of liver disease or portal hypertension (encephalopathy, asterixis, caput medusae, distended abdominal veins, spider nevi, gynecomastia, ascites)

  • Evaluation for chronic/excessive alcohol use (parotid gland enlargement, palmar erythema, Dupuytren’s contractures)

  • Evaluation for stigmata of endocarditis (conjunctival petechiae, Roth’s spots, splinter hemorrhages, Janeway lesions, Osler’s nodes)

  • Palpation of the liver including measure for hepatomegaly

  • Palpation of the abdomen for other masses

  • Heart auscultation for murmur or gallop

  • Auscultation of the lungs

  • Measurement of jugular venous pressure and check for hepatojugular reflux

  • Check for peripheral edema

  • Skin and peripheral extremity examination for temperature

  • Skin examination for rashes or nodules

  • Skin and mucosal examination for petechiae or purpura

  • Musculoskeletal examination for joint swelling or tenderness

3. Laboratory, radiographic and other tests that are likely to be useful in diagnosing the cause of this problem.

The following is a comprehensive list of potential testing (testing should be tailored to clinical suspicion):

  • Hemoglobin electrophoresis

  • Malaria thick and thin prep blood film

  • Examination for Schistosoma eggs in feces, urine, scrapings, or biopsy

  • Giemsa-stain identifying Leishmania organisms

  • Schistosoma serology

  • Antinuclear antibody

  • Complete blood count with differential

  • Peripheral blood smear

  • Reticulocyte count

  • Direct antiglobulin test

  • Coagulation profile

  • Comprehensive metabolic panel including liver function tests, protein, albumin

  • Erythrocyte sedimentation rate

  • C-reactive protein

  • Lactate dehydrogenase

  • B-type natriuretic peptide (BNP) or amino-terminal pro-BNP

  • Vitamin B12

  • Red blood cell folate

  • Serum protein electrophoresis

  • Urine protein electrophoresis

  • Blood cultures

  • Sputum or other body fluid acid fast bacilli smear/culture/polymerase chain reaction (PCR)/gene probe

  • Tuberculin skin test

  • Hepatitis B viral serologies

  • Hepatitis C virus antibody or viral load

  • Monospot test

  • Human immunodeficiency virus serologic testing and viral load

  • Cytomegalovirus serology

  • Toxoplasmosis serology or identification of Toxoplasma gondii organisms from fluid/tissue

  • Brucella serology

  • Typhus testing

  • Rapid plasma reagin (RPR)

  • Venereal Disease Research Lab (VDRL) test

  • Treponema pallidum antibody (Fluorescent Treponemal Antibody-Absorption or FTA-Abs)

  • Darkfield microscopy examination of fluid/tissue

  • Rheumatoid factor

  • Abdominal ultrasound

  • Abdominal computed tomography

  • Chest X-ray

  • Urinalysis with microscopy

  • Echocardiography

  • Heart catheterization

  • Chest/abdomen/pelvis computed tomography

  • Liver biopsy

  • Bone marrow biopsy with immunohistochemistry and flow cytometry analysis

  • Splenic fine-need aspiration

  • Splenic core biopsy

  • Diagnostic splenectomy

C. Criteria for Diagnosing Each Diagnosis in the Method Above.

Pertinent history and physical examination findings can help narrow the differential or pinpoint the underlying diagnosis or associated cause of splenomegaly. In many cases, additional diagnostic testing is needed to determine the cause of splenomegaly and differentiate between other possible causes. As mentioned above, splenectomy may be pursued in some cases when the underlying cause of splenomegaly remains undiagnosed after extensive testing.

Outlined below are some tests and criteria for diagnosing certain disorders. Splenomegaly is a common finding associated with a broad range of many important diseases; thus, additional review of those disorders and diagnostic testing may be found in chapters corresponding with the particular disease.

1. Lymphomas

  • Peripheral blood smear

  • Bone marrow biopsy with immunohistochemistry and flow cytometry

2. Leukemias

  • Peripheral blood smear

  • Bone marrow biopsy with immunohistochemistry and flow cytometry

3. Hairy cell leukemia

  • Peripheral blood smear

  • Bone marrow biopsy with immunohistochemistry and flow cytometry

4. Chronic myelogenous leukemia

  • Markedly elevated white blood cell count with left shift in myeloid series and low percentage of promyelocytes and blasts

  • Presence of Philadelphia chromosome or bcr-abl gene on analysis of peripheral smear or bone marrow biopsy

5. Primary myelofibrosis

  • Characteristic findings on peripheral blood smear suggesting bone marrow fibrosis

  • Bone marrow biopsy showing hypercellularity and fibrosis

  • In many cases of myelofibrosis the bone marrow may be difficult to aspirate ("dry tap")

6. Polycythemia vera

  • Increased hemoglobin/hematocrit and red cell mass on complete blood count

  • Normal arterial oxygen saturation

  • Other myeloproliferative disorders must also be ruled out

7. Essential thrombocytosis

  • Elevated platelet count in absence of other disorders (e.g. platelets as an acute phase reactant in certain disorders such as infection)

  • Normal red blood cell mass

  • Absence of Philadelphia chromosome

8. Hereditary spherocytosis

  • Spherocytes and increased reticulocytes on peripheral blood smear

  • Positive family history

  • Complete blood count/Iron indices consistent with microcytosis and hyperchromia

9. Thalassemias

  • Characteristic findings on peripheral blood smear

  • Positive family history

  • Evidence of hemolytic anemia

  • Hemoglobin electrophoresis

10. Hemoglobin SC Disease

  • Characteristic findings on peripheral blood smear

  • Positive family history

  • Evidence of hemolytic anemia

  • Hemoglobin electrophoresis

11. Autoimmune hemolysis

  • Evidence of hemolytic anemia

  • Positive direct antiglobulin test

  • Megaloblastic anemia

  • Characteristic findings on peripheral blood smear

  • Vitamin B12 or folate deficiency

  • Evidence of hemolytic anemia

12. Paroxysmal nocturnal hemoglobinuria

  • Episodic hemoglobinuria (by history or urinalysis)

  • Anemia

  • Elevated lactate dehydrogenase

  • Absence of CD59 on flow cytometry of bone marrow biopsy

13. Infectious mononucleosis

  • Positive Monospot (heterophile agglutination test)

  • Atypical large lymphocytes on peripheral blood smear

14. Viral hepatitides

  • Positive hepatitis serologies and viral ribonucleic acid/deoxyribonucleic acid (RNA/DNA) testing

15. Cytomegalovirus

  • Isolation of virus by culture or tissue

  • Positive cultures or confirmation of infectious source

16. Sepsis

  • Clinical criteria of sepsis

  • Positive cultures or confirmation of infectious source

17. Splenic abscess

  • Confirmation by imaging

  • Splenic biopsy/aspiration showing organisms or positive tissue culture

  • Positive blood culture of characteristic organism

18. Typhoid

  • Typhus antibody by complement fixation, microagglutination, or immunofluorescence

19. Toxoplasmosis

  • Serologic testing for toxoplasmosis (immunoglobulin G [IgG], immunoglobulin M [IgM])

  • Identification of T. gondii from blood/body fluids or in tissue/cytologic preparations.

20. Subacute bacterial endocarditis

  • Duke Criteria of clinical findings where 2 major, 1 major plus 3 minor, or 5 minor criteria must be fulfilled to improve diagnostic accuracy

  • Major criteria include two blood cultures positive for typical organisms causing endocarditis and echocardiographic findings of endocarditis or new regurgitant heart murmur on exam

  • Minor criteria include predisposing conditions for endocarditis, fever, evidence of embolic disease of endocarditis, immunologic phenomena of endocarditis, positive blood cultures not meeting major criteria, and echocardiographic findings of endocarditis not meeting major criteria

21. Mycobacterium tuberculosis

  • Positive acid fast bacillus on sputum or other body fluid stain/culture/PCR or gene probe for Mycobacterium tuberculosis

  • Positive tuberculin skin test and signs/symptoms of active tuberculosis

  • Characteristic pulmonary infiltrates on chest imaging and signs/symptoms of active tuberculosis

22. Brucellosis

  • Positive blood cultures for Brucella species

  • Serologic testing for Brucella species

23. Syphilis

  • Positive serologic testing including rapid plasma reagin (RPR),Venereal Disease Research Lab (VDRL) test,Treponema palladum antibody (Fluorescent Treponemal Antibody-Absorption or FTA-Abs)

  • Darkfield microscopic examination showing Treponema pallidum

24. Human immunodeficiency virus

  • Positive HIV serology and viral load

25. Malaria

  • Thick or thin prep blood films identifying parasites

  • History of exposure in malaria-endemic areas

26. Leishmaniasis (Visceral)

  • Biopsy with Giemsa-stain showing Leishmania organisms

27. Schistosomiasis

  • Schistosoma eggs identified in feces, urine, scrapings, or biopsy

  • Positive serology for Schistosoma

28. Cirrhosis

  • Characteristic findings on liver imaging

  • Liver biopsy

  • Abnormal liver function tests

  • Physical exam stigmata of liver disease

29. Portal/hepatic/splenic vein thrombosis

  • Liver/splenic imaging including magnetic resonance angiography (MRA)

30. Heart failure

  • Echocardiography

  • Cardiac catheterization with left heart ventriculography and measurements of pressure including of the right heart and pulmonary vasculature

  • History and physical exam findings consist with the clinical syndrome of heart failure

  • Abnormal liver function tests and liver enzymes suggestive of congestive hepatopathy

  • Elevated B-type natriuretic peptide (BNP) or amino-terminal pro-BNP

  • Chest X-ray demonstrating cardiomegaly and evidence of pulmonary venous hypertension

31. Systemic lupus erythematosus

  • Criteria for the classification of SLE by fulfilling 4 out of the 11 characteristic history/physical exam/lab/imaging findings in SLE

32. Rheumatoid arthritis

  • Rheumatoid factor

  • Characteristic history and physical exam findings of rheumatoid arthritis

  • Characteristic radiologic findings of rheumatoid arthritis

  • Extraarticular manifestations of rheumatoid arthritis

33. Sarcoidosis

  • Characteristic findings on chest imaging

  • History/physical exam findings of Sarcoidosis

34. Hemangiomas

  • Splenic imaging

  • Splenic biopsy

35. Metastatic disease (see neoplasms)

  • Splenic imaging

  • Splenic biopsy

36. Amyloidosis

  • Positive serum/urine protein electrophoresis

  • Skin/tissue biopsy findings characteristic of amyloidosis

37. Gaucher’s disease

  • Glucocerebrosidase activity deficiency in leukocytes

38. Splenic cysts

  • Splenic imaging

  • Splenic biopsy

39. Hematomas

  • Splenic imaging

  • Splenic biopsy

D. Over-utilized or “wasted” diagnostic tests associated with the evaluation of this problem.

  • Serum angiotensin converting enzyme (ACE)-level in diagnosing sarcoidosis

  • Anti-cyclic citrullinated peptide in diagnosing rheumatoid arthritis (this is a highly specific test and should not be used as a screening test for rheumatoid arthritis unless there is a high suspicion of disease)

  • Abdominal magnetic resonance imaging (MRI)/MRA in confirming splenomegaly

III. Management while the Diagnostic Process is Proceeding

A. Management of splenomegaly.

Splenomegaly by itself does not represent a medical emergency unless a patient is having acute complications or is medically unstable due to the effects of the splenomegaly. Splenomegaly is an important associated finding in many diseases that require prompt diagnosis and treatment. Some diseases, such as endocarditis, sepsis, and acute leukemia carry a high mortality rate in rapid diagnosis and treatment is not achieved.

Likewise, diseases causing acute fulminant liver failure are important to identify and treat early, although management is usually supportive in means unless liver transplant is considered. Acute decompensated heart failure may require a higher level of intensive or critical care. Likewise, acute heart failure due to ischemia/infarction or acute valvular insufficiency may require emergent percutaneous or surgical intervention.

Lymphomas, chronic leukemias and myeloproliferative diseases are important to identify and treat early, but diagnosis may be delayed in these conditions since the presenting signs and symptoms of these diseases are often non-specific. This holds true with recurrent or metastatic cancer, which may be difficult to diagnose at early stages. These patients often present when they become symptomatic from the cancer, which at that time typically represents advanced or end-stage disease, depending on the type of cancer.

Other diseases such as infectious mononucleosis may be self-limited if no complications arise. Many of the chronic infectious or inflammatory diseases causing splenomegaly are not necessarily urgent or emergent to treat, but it is important that a diagnosis is made and that long-term management is achieved in these patients to prevent further or future complications, morbidity and death.

While treating the underlying cause, once it is discovered, generally improves any symptoms of the splenomegaly, there are cases where partial splenic embolization may be considered. This procedure was initially designed to treat hypersplenism symptoms, but its utility has since expanded for use in controlling bleeding in splenic injury, improving platelet count in idiopathic thrombocytopenic purpura, and improving red blood cell counts in Thalassemia, hereditary spherocytosis, and autoimmune hemolytic anemia. Indications for partial embolization continue to grow, but the procedure is not without complications including infection, post-embolization syndrome, and pulmonary complications such as atelectasis, effusion, and pneumonia.

In general, the management of splenomegaly is focused on determining the underlying cause. In cases where the initial evaluation is unrevealing or unable to narrow the differential, careful thought must be had to determine the next steps in management. These undiagnosed patients are often considered to have isolated splenomegaly unless a cause is later found. While occult lymphoma remains a common and important concern in these undiagnosed patients, the further management of these patients typically involves three strategies depending on the degree of clinical suspicion for serious or life-threatening disease, as well as the patient’s preferences: watchful waiting, splenic biopsy and splenectomy.

B. Common Pitfalls and Side-Effects of Management of this Clinical Problem

While the management of many of the associated disorders for splenomegaly carry substantial risks/benefits, depending on the diagnosis and treatment, this portion of the text will address the important risk/benefits in the management of patients with splenomegaly who remain undiagnosed with regards to underlying cause after extensive laboratory and radiologic evaluation.

Watchful waiting

This approach is commonly taken with young, asymptomatic patients with a recent history of infection, despite no clear documentation at the time of work-up. The presumed cause is usually an undiagnosed, self-limited, viral illness. It is very important that these patients are truly symptom free, particularly of B symptoms including fevers, night sweats and weight loss which could be indicative of lymphoma or some chronic infection.

Likewise, the degree of splenomegaly should be mild in these patients. Complete blood counts should be mostly normal, although mild cytopenias or even reactive leukocytosis or thrombocytosis are commonly seen following acute viral infections. These patients should be followed closely for evidence of further splenic enlargement, new signs or symptoms heralding serious or a more chronic underlying illness, or persistent or worsening blood count abnormalities.

Bone marrow biopsy

Bone marrow biopsy is commonly performed in patients undergoing evaluation for splenomegaly. The purpose of this procedure is to determine or rule out if a hematologic dysplasia exists. This procedure is generally well-tolerated and considered a safe procedure. Common side effects of this procedure include pain or discomfort during the biopsy or at the biopsy site following the procedure. Minor bleeding at the puncture site is common.

Serious bleeding and infection are rare complications of this procedure. Bone marrow biopsy is typically performed at the patient’s bedside by specialists in hematology-oncology. This procedure may also be done in the office or outpatient procedure room; some cases require image-guided assistance used by interventional radiologists in order to obtain a successful biopsy. Consultation with a hematologist-oncologist should be obtained prior to performing bone marrow biopsy in the work-up of splenomegaly.

Splenic biopsy

The spleen is an organ of the lymphatic system and also functions to remove red blood cells and/or store blood cells. Despite misconceptions that biopsy of the spleen is not possible and not safe due to the "vascular" nature of the spleen, splenic biopsy is actually quite safe. The rate of major complications is similar to that of a biopsy of any other solid intra-abdominal organ. Biopsy should be considered in patients if the underlying cause of splenomegaly remains undiagnosed, if there are focal splenic lesions, if there is a high suspicion for abscesses, or if the patient is unwilling to undergo splenectomy.

An important consideration in splenic biopsy is the high false negative rate. Thus, splenic biopsy may obtain or confirm a diagnosis, but a negative or non-diagnostic result does not necessarily exclude serious underlying disease. Complications of splenic biopsy include bleeding, pain, pneumothorax, and complications leading to splenectomy.

There are two techniques available for splenic biopsy: fine-needle aspiration and core biopsy.

Fine-needle aspiration of the spleen is best suited for patients with focal splenic lesions where there is a chance to distinguish between benign lesions and malignant disease. Fine-needle aspiration may be less useful in patients with diffuse splenomegaly, but it does have a better safety profile than biopsy.

While core biopsy of the spleen carries slightly more risk than fine needle aspiration, it has improved accuracy in diagnosis. Current evidence suggests core biopsy may be the better technique when lymphoma is suspected as the cause of splenomegaly.

Despite the above information supporting splenic biopsy as a possible safe diagnostic procedure in the appropriate clinical setting, splenic biopsy remains a rarely performed procedure in most countries and is not currently recommended and/or practiced at most clinical institutions.

Splenectomy

Diagnostic splenectomy may be required in some patients, particularly those who remain undiagnosed after undergoing extensive evaluation as mentioned above. The diagnostic yield of splenectomy in determining the underlying cause of splenomegaly is much better than the above techniques but there is still a considerable number of cases that remain non-diagnostic. It should be noted that elective splenectomy is most often performed for therapeutic benefit for a condition typically not malignant in nature. In fact, splenectomy often reverses cytopenias present when functional hypersplenism exists.

While complication rates and perioperative mortality has improved over time, splenectomy still carries a significant risk for morbidity and mortality. Besides typical complications associated with intraabdominal surgery, splenectomy is associated with an increased risk of overwhelming sepsis due to encapsulated organisms (and patients should receive preoperative vaccinations against these organisms prior to splenectomy if possible). The risk-benefit ratio of splenectomy is quite small and should only be considered in patients who have undergone a non-diagnostic splenic biopsy, have a contraindication to splenic biopsy, or have severe symptoms or cytopenias in which diagnosis is critical.

What’s the evidence?

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