Dr Vishal Dublish, Dr Ira Shah
|It is also called as big spleen disease. Recently it has been redefined and more appropriately termed as " hyperreactive malarial syndrome" (HMS ).
Etiology: Although malarial parasite is usually not demonstrated in blood smears of patients with HMS, there are convincing evidence linking it to malaria:
Other factors which may play role in development of HMS include genetic susceptibility, malnutrition and pregnancy.
Pathogenesis: Exact mechanism is unknown. Evidence suggests that interaction of repeated malarial infections and unknown host factors leads to production of cytotoxic IgM anti-suppressor lymphocyte (CD8+) antibodies. This leads to exaggerated stimulation of polyclonal B lymphocytes causing excessive and uncontrolled production of IgM (which is polyclonal and not specific for any one particular malaria species) and formation of cryoglobulins (IgM aggregates and immune complexes). These macromolecular aggregates get deposited in Kupffer cells in liver and spleen leading to reticuloendothelial hyperplasia and hepatosplenomegaly (massive and progressive) leading to anemia.
- It is prevalent only in malaria endemic areas.
- High levels of malarial antibodies can be demonstrated in patients with HMS.
- Anti malarial treatment reduces splenic size suggesting response to treatment.
- Discontinuation of treatment prematurely may be associated with relapse of HMS.
- Effective malarial chemoprophylaxis has been shown to reduce spleen rates as well as incidence of HMS.
- Sickle cell trait patients are relatively protected from HMS as with malaria.
- Animal models have been developed to show similar clinical syndrome after malarial infection.
- Age: More frequent in young and middle aged adults. Uncommon in children younger than 8 years, suggesting that prolonged chronic antigenic stimulation is an important factor in development of HMS.
- Sex: More common in females, male : female = 1:2.
Abdominal distention - waxing and waning.
Chronic, dragging pain sensations
Fever is rare, if present it suggests different diagnosis.
Hernia, leg swellings
Skin and respiratory tract infections
Bleeding episodes - very rare
Splenomegaly (moderate to massive & firm and regular). It is the hallmark of HMS.
Splenic bruit may be present
Pallor (without tachycardia)
Abdominal vein dilatation
Cardiomegaly, flow murmurs because of hypervolemia.
Jaundice may be present, Ascitis uncommon.
Lymphadenopathy absent, parotid enlargement may be seen.
Sickle cell anemia, Thalassemia
Leukemia, lymphoma, myelofibrosis
Anemia (normocytic normochromic). Coombs test is negative
Peripheral smear - malarial parasite not detectable. Increased reticulocyte count.
IgM against malaria increased >2 standard deviation above local mean.
High titers of cold agglutinins, rheumatoid factor, antinuclear factor and cryoproteins.
Phytohemagglutination stimulation test - to differentiate HMS from lymphomas and chronic lymphocytic leukemia.
Hepatic sinusoidal lymphocytosis
No malarial pigmentation in macrophages.
Spleen - Kupffer cell hypertrophy and hyperplasia.
Infections especially of skin and gastrointestinal tract
Predisposition to development of malignancy
Congestive cardiac failure.
Criteria for diagnosis
- Gross splenomegaly
- Presence of antimalarial antibodies
- Clinical and immunologic response to antimalarials.
- Hepatic sinusoidal lymphocytosis
- Normal cellular and humoral responses to antigenic challenge
- Normal phytohemagglutination response
- Lymphocytic proliferation
- Familial occurrence.
|How to Cite URL :|
|Shah I D, Dublish V D. TROPICAL SPLENOMEGALY SYNDROME. Pediatric Oncall [serial online] 2006[cited 2006 January 1];3. Art #24. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=777&type=J&tid=&imgid=&reportid=369&tbltype=|