Case of the Month - October 2011
Answer to Q1
- Malaria (Hyper-reactive malarial splenomegaly) – most likely
- Schistosomiasis (Serology not suggestive, but no rectal snip etc. done)
- Gaucher Disease (but bone marrow not suggestive)
- Kala-Azar (Visceral Leishmaniasis) – from incorrect geographical area
- CML (unusual age)
- Haemophagocytic Syndrome – (BMAT not suggestive)
Question 2: What additional blood test(s) would have confirmed hyperreactive malarial splenomegaly?
Answer to Q2
- Hyper-reactive tropical splenomegaly used to be known as “tropical splenomegaly”
- The splenomegaly is distinct from splenomegaly associated with malarial parasitaemia.
It is thought to be due to a disturbance in the T-lymphocyte control of the humoral response to recurrent
malaria (?related to HLA Class H antigens)
This leads to the gross overproduction of IgM antibodies which leads to the formation of high molecular weight
immune complexes, persistent gross splenomegaly, recurrent episodes of profound anaemia and an increased
susceptibility to infections (bacterial and viral).
Typical lab findings:
- Very high IgM
- High malarial antibody titres
- Malarial parasites are NOT seen during the acute episodes
Question 3: What are the diagnostic criteria for hyperreactive malarial splenomegaly?
Answer to Q3
- Gross splenomegaly (10 cm or more below the costal margin in adults) for which no other cause can be found
- Elevated serum IgM level (2 standard deviations or more above the local mean)
- Clinical and immunologic responses to antimalarial therapy
- Regression of splenomegaly by 40% by 6 months after start of therapy
- High antibody levels of Plasmodium species (≥ 1:800) – this test is not routinely offered by NHLS
- Hepatic sinusoidal lymphocytosis
- Normal cellular and humoral responses to antigenic challenge
- Lymphocytic proliferation
- Familial occurrence
Question 4: What are the possible causes for her nephrotic syndrome?
Answer to Q4
- HIV-associated nephropathy (usually FSGS)
- Related to Plasmodium malariae coinfection (usually membranous glomerulonephritis)
- Hepatitis B
- Some malignancies
Hyper-reactive Malarious Splenomegaly (Tropical Splenomegaly Syndrome); G.G. Crane; Parastitology Today,
vol. 2, no. I, 1986
Case Report: Hyper-reactive Malarial Splenomegaly in a Patient with Human Immunodeficiency Virus; G. De Iaco,
N. Saleri et al; Am. J. Trop. Med. Hyg., 78(2), 2008, pp. 239–240
- Fakunle Y.Tropical splenomegaly. In: Luzzatto L, ed. Clinics in haematology.London:WB Saunders, 1981: 963–75
This tragic case illustrates the importance of considering non-HIV related pathology in HIV-infected patients. In
addition, it demonstrates the difficulty in diagnosing this condition particularly as widely available malaria tests
are typically negative. Finally, this case highlights the value of post-mortem evaluation when patients die of
incompletely explained causes.