HMSS- hyper reactive malarial splenomegaly syndrome, is one of the chronic form of malaria. it commonly occurs in peoples from malaria endemic areas who have recurrent malarial attacks.
It is an abnormal immune response after frequent malaria infections in existing semiimmunity. Hyperreactive malaria splenomegaly predominantly occurs in adolescents and adults (women> men) and only in endemic regions. The picture is characterized by an often extreme splenomegaly, hepatomegaly, massive elevation of IgM antibodies and lymphocytosis in peripheral blood. In addition, there are normochromic / normocytic anemia, signs of hypersplenism, hypervolemia, fever attacks and abdominal complaints; Parasites are undetectable. The syndrome appears to predispose to B-cell splenic lymphoma with villous lymphocytes.
1. Gross splenomegaly 10 cm or more below the costal margin in adults for which no other cause can be found
2. Elevated serum IgM level 2 standard deviations or more above the local mean 3. Clinical and immunologic responses to antimalarial therapy
4. Regression of splenomegaly by 40% by 6 months after start of therapy
5. High antibody levels of Plasmodium species (≥ 1:800)
Minor criteria include the following:
1. Hepatic sinusoidal lymphocytosis
2. Normal cellular and humoral responses to antigenic challenge, including a normal phytohemagglutination response
3. Hypersplenism
4. Lymphocytic proliferation
5. Familial occurrence
It is possible that some of the cases with negative blood films and QBC would have been found positive by the more sensitive PCR. It has recently been suggested by McGregor et al. [1] that subjects with a positive PCR would be more likely to respond to ther-apy than negative subjects, indicating a role of molecular diagnosis in the case definition of the syndrome.
[1]Article Chronic malaria and hyper-reactive malarial splenomegaly: A ...