Tropical Splenomegaly Syndrome Clinical Presentation

  • Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: May 9, 2012
 

History

  • The most common presenting symptoms of hyperreactive malarial syndrome (HMS), or tropical splenomegaly syndrome, are chronic abdominal swelling (64%) and pain (52%).[3] Abdominal swelling may wax and wane. Some patients present with acute abdominal pain. Pressure on the abdominal contents may also lead to hernias and leg swelling.
  • A history of chronic splenic enlargement differentiates HMS from simple malarial splenomegaly.
  • Almost all patients (97%) report weight loss.
  • Patients physiologically adapt well to the chronic evolution of anemia and are symptomatic only when anemia is severe. Many patients do not have any symptoms and are capable of normal daily activity. Weakness, loss of energy, and severe headache may signify severe anemia.
  • Rarely, patients have intermittent fever. Persistent, severe fevers should raise the possibility of an alternative diagnosis.[20]
  • Bleeding complications such as epistaxis are uncommon because thrombocytopenia is usually not severe.
  • Susceptibility to infections, especially skin and respiratory infections, is slightly increased.
  • Pregnant women are susceptible to episodes of massive Coombs-negative hemolysis, which are usually preceded by febrile episodes. Coombs-positive hemolysis is associated rarely with acute attacks of plasmodium vivax malaria.[21]
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Physical

  • Patients are usually afebrile at presentation. Pallor is common, and the patient may be malnourished and jaundiced.
  • In general, tachycardia is absent. If tachycardia is present, it indicates a concurrent complication.
  • The hallmark of HMS is splenomegaly, which is usually moderate to massive. Most spleens (63%) are not tender, and almost all have a smooth surface, soft consistency, and sharp border.[19] The enlarged spleen may be seen to protrude against the abdominal wall, and a splenic bruit may be audible. Despite the size of the spleen, splenic rupture is rare.
  • Hepatomegaly is common; in a study of 69 Nigerian patients, 93% had accompanying hepatomegaly.[3] Ascites is uncommon.
  • Dilatation of the veins, cardiomegaly, low blood pressure, and flow murmurs reflect hypervolemia.
  • Lymphadenopathy is absent, but bilateral parotid swelling has been described.
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Causes

  • The most important predisposing factor for HMS is residence in or visitation to an area where malaria is endemic.
  • Other risk factors include malnutrition and an as-yet-undefined genetic predisposition.
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Contributor Information and Disclosures
Author

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP  Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Royal College of Physicians of the United Kingdom

Disclosure: Nothing to disclose.

Coauthor(s)

Mundeep K Kainth, DO  Resident Physician, Department of Pediatrics, The Children's Hospital at Albany Medical Center

Mundeep K Kainth, DO is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

J Martin Johnston, MD  Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Mudra Kumar, MD, MBBS, to the original writing and development of this article.

References

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  2. Bryceson A, Fakunle YM, Fleming AF, et al. Malaria and splenomegaly. Trans R Soc Trop Med Hyg. 1983;77(6):879. [Medline].

  3. Fakunle YM. Tropical splenomegaly. Part 1: Tropical Africa. Clin Haematol. Oct 1981;10(3):963-75. [Medline].

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  8. Piessens WF, Hoffman SL, Wadee AA, et al. Antibody-mediated killing of suppressor T lymphocytes as a possible cause of macroglobulinemia in the tropical splenomegaly syndrome. J Clin Invest. Jun 1985;75(6):1821-7. [Medline]. [Full Text].

  9. Singh RK. Hyperreactive malarial splenomegaly in expatriates. Travel Med Infect Dis. Jan 2007;5(1):24-9. [Medline].

  10. Lowenthal MN, Hutt MS, Jones IG, Mohelsky V, O'Riordan EC. Massive splenomegaly in Northern Zambia. I. Analysis of 344 cases. Trans R Soc Trop Med Hyg. 1980;74(1):91-8. [Medline].

  11. Bedu-Addo G, Bates I. Causes of massive tropical splenomegaly in Ghana. Lancet. Aug 10 2002;360(9331):449-54. [Medline].

  12. Pryor DS. Tropical splenomegaly in New Guinea. Q J Med. Jul 1967;36(143):321-36. [Medline].

  13. Crane GG, Wells JV, Hudson P. Tropical splenomegaly syndrome in New Guinea. I. Natural history. Trans R Soc Trop Med Hyg. 1972;66(5):724-32. [Medline].

  14. Fakunle YM, Greenwood BM. Mortality in tropical splenomegaly syndrome. Trans R Soc Trop Med Hyg. 1980;74(3):419. [Medline].

  15. Bates I, Bedu-Addo G, Bevan DH, Rutherford TR. Use of immunoglobulin gene rearrangements to show clonal lymphoproliferation in hyper-reactive malarial splenomegaly. Lancet. Mar 2 1991;337(8740):505-7. [Medline].

  16. De Iaco G, Saleri N, Perandin F, et al. Hyper-reactive malarial splenomegaly in a patient with human immunodeficiency virus. Am J Trop Med Hyg. Feb 2008;78(2):239-40. [Medline]. [Full Text].

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  18. Van den Ende J, van Gompel A, van den Enden E, et al. Hyperreactive malaria in expatriates returning from sub-Saharan Africa. Trop Med Int Health. Sep 2000;5(9):607-11. [Medline].

  19. Allam MM, Alkadarou TA, Ahmed BG, et al. Hyper-reactive Malarial Splenomegaly (HMS) in malaria endemic area in Eastern Sudan. Acta Trop. Feb 2008;105(2):196-9. [Medline]. [Full Text].

  20. Verma S, Aggarwal A. Hyper-reactive malarial splenomegaly: rare cause of pyrexia of unknown origin. Indian J Pediatr. Apr 2007;74(4):409-11. [Medline].

  21. Mitjà O, Hays R, Malken J, Ipai A, Kangapu S, Robson J. HMS-related hemolysis after acute attacks of Plasmodium vivax malaria. Am J Trop Med Hyg. Oct 2011;85(4):616-8. [Medline].

  22. Torres JR, Villegas L, Perez H, et al. Low-grade parasitaemias and cold agglutinins in patients with hyper-reactive malarious splenomegaly and acute haemolysis. Ann Trop Med Parasitol. Mar 2003;97(2):125-30. [Medline].

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  25. [Best Evidence] Aponte JJ, Aide P, Renom M, et al. Safety of the RTS,S/AS02D candidate malaria vaccine in infants living in a highly endemic area of Mozambique: a double blind randomised controlled phase I/IIb trial. Lancet. Nov 3 2007;370(9598):1543-51. [Medline].

 
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Young patient with hepatomegaly and massive splenomegaly.
 
 
 
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