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Splenomegaly Workup

  • Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Apr 29, 2016
 

Approach Considerations

Clinically detected splenomegaly is confirmed and quantified using imaging studies. Ultrasonography is a noninvasive, highly sensitive, and specific imaging technique for the evaluation of splenic size. Point-of-care ultrasonography significantly improved the sensitivity of physical examination alone in diagnosing splenomegaly in a prospective study of 39 adult hospitalized patients.[11]

In imaging studies, a craniocaudal measurement of 11-13 cm is frequently used as the upper limit of normal splenic size. However, because of wide variations in shape, no consistent correlation has been recognized between the spleen's length and its overall volume, as has been determined for other organs (eg, kidney).

Angiographic findings are used to differentiate splenic cysts from other splenic tumors. Splenoportography is used to evaluate portal vein patency and the distribution of collateral vessels before shunt operations for cirrhosis. Splenoportographic findings can help to identify the cause of idiopathic splenomegaly, especially in children.

Perform a complete blood count (CBC) with differential, platelet count, and peripheral blood smear in cases of splenomegaly. These studies can identify sickle cell disease, spherocytosis, and other hereditary hemolytic anemias.

If the differential count reveals a lymphocyte predominance, flow cytometry should be performed. Results consistent with neoplasm may prompt fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) testing for BCR-ABL or Jak 2. Depending on the apparent etiology, bone marrow biopsy may be needed.

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Findings in Hypersplenism

The term hypersplenism describes some of the sequelae that are often observed with splenomegaly. Criteria for a diagnosis of hypersplenism include anemia, leukopenia, thrombocytopenia, or combinations thereof, plus cellular bone marrow, splenomegaly, and improvement after splenectomy.

Anemia

The anemia observed in splenomegaly results from sequestration and hemodilution.

Leukopenia

Increased destruction or sequestration of leukocytes causes the leukopenia observed in splenomegaly. Leukopenia is closely related to neutropenia. Neutropenia (absolute neutrophil count [ANC] <1800/μL) is the result of an increase in the marginated granulocyte pool, a portion of which is located in the spleen (see the Absolute Neutrophil Count calculator). Sequestration may also play a role in the genesis of neutropenia.

Thrombocytopenia

Approximately 30% of the total platelet mass exists as an exchangeable pool in the spleen. Increased splenic platelet pooling is the primary cause of the thrombocytopenia of hypersplenism. In patients with hypersplenism, as much as 90% of the total platelet mass can be found in the spleen. In hypersplenism, the platelet count is usually 50,000-150,000/µL.

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CT Scanning

The underlying histologic anatomy of the spleen largely determines its characteristic appearance on abdominal computed tomography (CT) scans. On unenhanced CT scans, the spleen has an attenuation similar to that of the liver, approximately 40 Hounsfield units (H). Normally, the liver and spleen densities are within 25 H on dynamic contrast-enhanced CT scans.[12]

In general, the spleen can be considered enlarged if its craniocaudal length is more than 10cm on conventional CT scans. A spleen that extends below the lower third pole of the kidney is also indicative of splenomegaly.

A CT scan remains the most useful preoperative investigation to measure splenic volume; to exclude lymph nodes at the splenic hilum; and to detect accessory spleens, splenic abscesses, and perisplenitis.

Findings that indicate radiologic distinction between benign and malignant lesions are inconsistent and cannot be relied on to establish or refute a diagnosis of malignancy.

CT scanning is the imaging study of choice for identification of inflammatory changes. In addition, CT scanning is sensitive for detecting mass lesions, calcifications, infarcts, and cysts.

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Liver-Spleen Colloid Scanning

Erythrocytes are labeled with chromium-51 (51Cr) , mercury-197 (197Hg), rubidium-81 (81Rb), or technetium-99m (99mTc), and the cells are altered by treatment with heat, antibody, chemicals, or metal ions so that the spleen sequesters them after infusion. A splenic length of greater than 14 cm is consider enlarged on liver-spleen scan

A spleen scan is a good noninvasive technique for evaluating splenic size; a close correlation exists between splenic length on the scan images and splenic weight after splenectomy.

A spleen scan is also useful for detecting space-occupying lesions in the splenic substance, evaluating loss of splenic functions, assessing for the absence of a spleen, or determining the presence of an accessory spleen.

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Splenectomy and Splenic Biopsy

History and physical examination, laboratory studies, and CT scanning can help clinicians to determine the etiology of splenomegaly in greater than 90% of cases. Occasionally, however, it is necessary to obtain splenic tissue for pathologic evaluation.

Splenectomy

Splenectomy may be considered in certain individuals to determine the etiology of splenomegaly.[13, 14, 15, 16, 17] However, the need for a diagnosis must be carefully weighed against the confounding morbidity associated with the asplenic state. Splenectomy is typically performed laparoscopically; even supramassive spleens can be removed by laparoscopic surgery with minimal morbidity.[17, 18] Splenectomy is therapeutic in individuals with severe pancytopenia due to splenomegaly.

Splenic biopsy

Splenic biopsy may be performed in specialized institutions. Severe bleeding is a frequent complication that limits the usefulness of this procedure.

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Histologic Findings

When referring to an enlarged spleen as hypertrophied, the underlying cause may be hypertrophy or hyperplasia of individual cells. In specific diseases, the splenic architecture is remodeled. For example, in Niemann-Pick disease, sphingomyelin and cholesterol accumulate within large foamy cells, which is characteristic of this disease.

With amyloidosis involving the spleen and resulting in splenomegaly, large hyaline masses are seen as lesions occupying the white pulp space. Two forms exist, including the "sago spleen," in which amyloid deposits are limited to follicles, and the "lardaceous spleen," in which amyloid is deposited in the walls of the splenic sinusoids. In a rare complication of typhoid fever, reactive splenic vasculitis may develop.

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Laboratory Studies

Laboratory studies include the following:

  • Complete blood cell count (CBC) with differential
  • Liver function testing
  • Hepatitis B and C testing
  • Lactate dehydrogenase (LDH)
  • Erythrocyte sediumentation rate (ESR)
  • Evaluation of peripheral blood smear for RBC morphology and signs of myeloproliferative disorders or underlying bone marrow disorders
  • Prothrombin time with international normalized ratio (INR) and activated partial thromboplastin time (aPTT)

 

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Contributor Information and Disclosures
Author

Neetu Radhakrishnan, MD Assistant Professor of Medicine, Division of Hematology/Oncology, University of Cincinnati Medical Center; Lab Director, Hematology Lab, University Point, West Chester

Neetu Radhakrishnan, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada, American Clinical and Climatological Association, International Society of Blood Transfusion

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,,Grifols<br/>Received honoraria from all the above companies for speaking and teaching.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Gina M Matacia-Murphy, MD Fellow in Hematology/Oncology, University of Cincinnati College of Medicine

Gina M Matacia-Murphy, MD is a member of the following medical societies: American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Acknowledgements

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

David Coffman, MD Fellow, Department of Surgery, Division of Trauma and Critical Care, Yale University School of Medicine

Disclosure: Nothing to disclose.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Emmanuel N Dessypris, MD Professor of Medicine, Medical College of Virginia; Chief, Medical Service, Hunter Holmes McGuire Department of Veterans Affairs Medical Center

Emmanuel N Dessypris, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society of Hematology, New York Academy of Sciences, Society for Experimental Biology and Medicine, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

David J Draper, MD Fellow, Department of Hematology/Oncology, The University Hospital, University of Cincinnati College of Medicine

Disclosure: Nothing to disclose.

Lewis J Kaplan, MD, FACS, FCCM, FCCP Director, SICU and Surgical Critical Care Fellowship, Associate Professor, Department of Surgery, Section of Trauma, Surgical Critical Care, and Surgical Emergencies, Yale University School of Medicine

Lewis J Kaplan, MD, FACS, FCCM, FCCP is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Surgeons, Association for Academic Surgery, Association for Surgical Education, Connecticut State Medical Society, Eastern Association for the Surgery of Trauma, International Trauma Anesthesia and Critical Care Society, Society for the Advancement of Blood Management, Society of Critical Care Medicine, and Surgical Infection Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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This patient has a splenic abscess due to pneumococcal bacteremia. Note that the massively enlarged spleen is readily visible, with minimal retraction in the left upper quadrant.
Resected specimen from the patient in the previous image. Note the discrete abscesses adjacent to normal parenchyma.
The margins of this massive spleen were palpated easily preoperatively. Medially, the 3.18 kg (7 lb) spleen crosses the midline. Inferiorly, it extends into the pelvis.
Massive splenomegaly does not preclude splenectomy through a minimally invasive approach. This photograph depicts a fragmented 3.2 kg (7.05 lb) spleen after removal via a hand-assisted laparoscopic technique.
A portion of a massive spleen is extracted via hand-assisted laparoscopy.
Intraoperative photograph of a laparoscopic splenectomy being taken down using the hanging-pedicle technique. The tip of the spleen is visualized in the background, whereas the stapler is detailed in the foreground across a segment of the pedicle.
A massive spleen that was removed from an elderly woman with lymphoma.
 
 
 
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