eMedicine Specialties > Hematology > Immune System and Disorders

Splenomegaly: Treatment & Medication

Author: David J Draper, MD, Hematology/Oncology Fellow, The University Hospital, Cincinnati, Ohio
Coauthor(s): Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center; Emmanuel N Dessypris, MD, Professor of Medicine, Medical College of Virginia; Chief, Medical Service, Hunter Holmes McGuire Department of Veterans Affairs Medical Center; 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
Contributor Information and Disclosures

Updated: Oct 4, 2009

Treatment

Medical Care

Successful medical treatment of the primary disorder in cases of splenomegaly can lead to regression of the hypersplenism without the need for surgery.

  • Chemotherapy is used for hematologic malignancies.
  • Antibiotics are used for infection, with the exception of that which is associated with a splenic abscess. This requires surgical intervention.
  • Immunosuppression is used for autoimmune or inflammatory disorders, treatment of cirrhosis, and CHF.
  • All patients scheduled for elective splenectomy (either diagnostic or therapeutic) should receive a pneumococcal vaccines. Also consider administering prophylaxis against Haemophilus influenzae and Neisseria meningitidis.

Surgical Care

  • Splenectomy is indicated to help control or stage the basic disease in cases of splenomegaly. These diseases can include hereditary spherocytosis, autoimmune thrombocytopenia or hemolysis, or Hodgkin disease (as part of a staging celiotomy).
  • Splenectomy is also indicated for the treatment of chronic, severe hypersplenism.11 This can occur in conditions such as hairy cell leukemia, Felty syndrome, agnogenic myeloid metaplasia, thalassemia major, Gaucher disease, hemodialysis splenomegaly, or splenic vein thrombosis.
  • In rare cases, splenectomy may be used to treat thrombotic thrombocytopenic purpura (TTP); therapeutic plasma exchange transfusion (plasmapheresis) has largely supplanted the need for splenectomy.
  • The vast majority of splenectomies are performed using laparoscopic techniques. Laparoscopic splenectomy is safe and is associated with reduced hospital stays. Furthermore, this procedure has a postoperative survival advantage when compared with open procedures. Laparoscopic surgery can be performed even on individuals with massive splenomegaly.12,13
  • Occasionally, a reactive thrombocytosis occurs following splenectomy. Thrombocytosis in the face of splenectomy rarely requires treatment. It is most common in patients with massive splenomegaly from myeloproliferative disorders.
  • An onset of fever several days following splenectomy can be due to a recrudescence of malaria.
    • This should be considered as a cause of fever in patients who have lived in areas commonly associated with malaria and in persons who abuse intravenous drugs who share needles.
    • With Plasmodium malariae infection, this may occur decades after the initial infection. Malaria from P vivax (3-7 y) and P falciparum (~1 y) remain active for shorter intervals after the initial infection.

Consultations

  • Consultation with a hematologist is ideal before surgery for enlarged spleens in order to secure necessary blood products. Postoperative management does not usually require intervention from a hematologist.

Activity

  • The usual postoperative activity restrictions imposed on a patient who has undergone a laparotomy or laparoscopy also apply to this patient population after splenectomy.
  • Patients with uncorrected splenomegaly should be counseled to refrain from contact sports or activities that would predispose them to blunt abdominal trauma. Examples include skydiving, horseback riding, soccer, football, and ice hockey, among other activities. These restrictions reduce the likelihood that blunt injury will lead to splenic rupture and uncontrolled hemorrhage.

Medication

The goals of pharmacotherapy in cases of splenomegaly are to reduce mortality and to prevent complications. Recall that in the absence of a functional spleen, patients have a defect in bacterial clearance due to impaired opsonization. In particular, these patients are at risk for overwhelming postsplenectomy sepsis due to infection with encapsulated organisms such as H influenzae, N meningitidis, and Streptococcus pneumoniae.14

Vaccines

Vaccines aid in the generation of an anamnestic response to invasion with the target organism.


Pneumococcal vaccine (Pneumovax 23)

Contains capsular polysaccharides of 23 pneumococcal types, which comprise 98% of pneumococcal disease isolates.

Adult

0.5 mL IM/SC

Pediatric

<2 years: Contraindicated

>2 years: Administer as in adults.

Effects decrease with immunosuppressive agents (eg, immunosuppressive doses of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents); globulin preparations may interfere with the immune response and reduce the efficacy (do not administer within 3 mo of vaccine)

Documented hypersensitivity to any component or to thimerosal; severe or moderate febrile illness; age <2 y; thrombocytopenia or coagulation disorder contraindicating IM injection (unless benefits outweigh risks)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause relapse in patients with stable idiopathic thrombocytopenia purpura; adverse effects include arthralgia, fever, urticaria, and Guillain-Barré syndrome (rarely)


Meningococcal vaccine, groups A, C, Y, and W-135 (Menomune-A/C/Y/W-135)

Capsular polysaccharide antigens (groups A, C, Y, and W-135) of N meningitidis. May be used to prevent and control outbreaks of serogroup C meningococcal disease according to CDC guidelines. Induces formation of bactericidal antibodies to meningococcal antigens. Used for active immunization against invasive meningococcal disease caused by inclusive serogroups. Vaccine induces antibody response for serogroup A in individuals as young as 3 mo, but it is poorly immunogenic for serogroup C in recipients younger than 18-24 mo.

Adult

0.5 mL SC

Pediatric

<2 years: Contraindicated

>2 years: Administer as in adults.

Administration of immunoglobulin within 1 mo or concurrent administration with immunosuppressive agents may inhibit full the immunologic response; coadministration with whole-cell pertussis or whole-cell typhoid vaccines may increase the endotoxin content.

Documented hypersensitivity; children <2 y; IV/IM/ID administration

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Asplenic patients with lymphoid tumors who receive either chemotherapy or irradiation respond poorly; avoid during the course of acute illness; routine vaccination is recommended for high-risk groups (eg, those with deficiencies in late complement components [C3, C5-C-9], personnel with laboratory or industrial exposure to N meningitidis aerosols, travelers or residents of hyperendemic areas); for information concerning the geographic areas in which vaccination is recommended, contact the CDC at (404) 332-4559; serious adverse reactions should be reported to the US Department of Health and Human Services at (800) 822-7967.


Haemophilus B conjugate vaccine (ActHIB, HibTITER, PedvaxHIB)

Used for the routine immunization of children against invasive diseases caused by H influenzae type B. Decreases nasopharyngeal colonization. The CDC Advisory Committee on Immunization Practices recommends all children receive a conjugate vaccine licensed for infant use at age 2 mo.

Adult

Not indicated

Pediatric

Regimens vary depending on the product.

Example for HibTITER

<2 months: Not established

2-6 months: 0.5 mL IM every 2 mo for 3 doses

7-11 months: Previously unvaccinated, 0.5 mL IM every 2 mo for 2 doses

12-14 months: Previously unvaccinated, 0.5 mL IM once

Booster dose: 0.5 mL at age 15 mo, or at least 2 mo after last dose of series; if age 15-71 mo and previously unvaccinated, 0.5 mL IM once

Immunoglobulins given within 1 mo or concurrent administration with immunosuppressive agents may inhibit the full immunologic response.

Documented hypersensitivity; immunosuppression in children or use of immunosuppressive therapy; IV/ID/SC administration

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Delay immunization if a febrile illness is evident; may cause local erythema, swelling, or tenderness; risk of Haemophilus B infections increases in the week after vaccination; a cause-and-effect relationship with observed postvaccine Guillain-Barré syndrome has not been established; serious adverse reactions should be reported to US Department of Health and Human Services at (800) 822-7967.

More on Splenomegaly

Overview: Splenomegaly
Differential Diagnoses & Workup: Splenomegaly
Treatment & Medication: Splenomegaly
Follow-up: Splenomegaly
Multimedia: Splenomegaly
References
Further Reading
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References

  1. Eichner ER. Splenic function: normal, too much and too little. Am J Med. Feb 1979;66(2):311-20. [Medline].

  2. Zhu JH, Wang YD, Ye ZY, Zhao T, Zhu YW, Xie ZJ, et al. Laparoscopic versus open splenectomy for hypersplenism secondary to liver cirrhosis. Surg Laparosc Endosc Percutan Tech. Jun 2009;19(3):258-62. [Medline].

  3. Anegawa G, Kawanaka H, Uehara H, Akahoshi T, Konishi K, Yoshida D, et al. Effect of laparoscopic splenectomy on portal hypertensive gastropathy in cirrhotic patients with portal hypertension. J Gastroenterol Hepatol. Sep 2009;24(9):1554-8. [Medline].

  4. Bezerra AS, D'Ippolito G, Faintuch S, Szejnfeld J, Ahmed M. Determination of splenomegaly by CT: is there a place for a single measurement?. AJR Am J Roentgenol. May 2005;184(5):1510-3. [Medline]. [Full Text].

  5. Goldstone J. Splenectomy for massive splenomegaly. Am J Surg. Mar 1978;135(3):385-8. [Medline].

  6. Laws HL, Burlingame MW, Carpenter JT, Prchal JT, Conrad ME. Splenectomy for hematologic disease. Surg Gynecol Obstet. Oct 1979;149(4):509-12. [Medline].

  7. Musser G, Lazar G, Hocking W, Busuttil RW. Splenectomy for hematologic disease. The UCLA experience with 306 patients. Ann Surg. Jul 1984;200(1):40-5. [Medline]. [Full Text].

  8. Wilhelm MC, Jones RE, McGehee R, et al. Splenectomy in hematologic disorders. The ever-changing indications. Ann Surg. May 1988;207(5):581-9. [Medline]. [Full Text].

  9. Flowers JL, Lefor AT, Steers J, et al. Laparoscopic splenectomy in patients with hematologic diseases. Ann Surg. Jul 1996;224(1):19-28. [Medline]. [Full Text].

  10. Wang KX, Hu SY, Zhang GY, Chen B, Zhang HF. Hand-assisted laparoscopic splenectomy for splenomegaly: a comparative study with conventional laparoscopic splenectomy. Chin Med J (Engl). Jan 5 2007;120(1):41-5. [Medline]. [Full Text].

  11. Subhasis RC, Rajiv C, Kumar SA, Kumar AV, Kumar PA. Surgical treatment of massive splenomegaly and severe hypersplenism secondary to extrahepatic portal venous obstruction in children. Surg Today. 2007;37(1):19-23. [Medline].

  12. Kawanaka H, Akahoshi T, Kinjo N, Konishi K, Yoshida D, Anegawa G, et al. Technical standardization of laparoscopic splenectomy harmonized with hand-assisted laparoscopic surgery for patients with liver cirrhosis and hypersplenism. J Hepatobiliary Pancreat Surg. Jul 22 2009;[Medline].

  13. Xu WL, Li SL, Wang Y, Shi BJ, Li M, Li YC, et al. Laparoscopic splenectomy: color Doppler flow imaging for preoperative evaluation. Chin Med J (Engl). May 20 2009;122(10):1203-8. [Medline].

  14. Shaw JH, Print CG. Postsplenectomy sepsis. Br J Surg. Oct 1989;76(10):1074-81. [Medline].

  15. Poulin EC, Mamazza J, Schlachta CM. Splenic artery embolization before laparoscopic splenectomy. An update. Surg Endosc. Jun 1998;12(6):870-5. [Medline].

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Further Reading

Related eMedicine Topics

Clinical Trial
 Clinical Guidelines

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Keywords

splenomegaly, enlarged spleen, splenectomy, ruptured spleen, hypersplenism, spleen enlargement, splenic enlargement, enlargement of the spleen, subacute bacterial endocarditis, SBE, infectious mononucleosis, hereditary spherocytosis, thalassemia major, splenic vein thrombosis, portal hypertension, chronic myeloid metaplasia, sarcoidosis, neoplasm, chronic lymphocytic leukemia, CLL, lymphoma,trauma, cyst, hemangioma, metastasis, giant abscess, tropical splenomegaly syndrome, hyperactive malarial syndrome, splenic injury 

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Contributor Information and Disclosures

Author

David J Draper, MD, Hematology/Oncology Fellow, The University Hospital, Cincinnati, Ohio
Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

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.

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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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