NEWS & EVENTS

Case of the Month - October 2011

Answer to Q1

  1. Malaria (Hyper-reactive malarial splenomegaly) – most likely
  2. Schistosomiasis (Serology not suggestive, but no rectal snip etc. done)
  3. Gaucher Disease (but bone marrow not suggestive)
  4. Kala-Azar (Visceral Leishmaniasis) – from incorrect geographical area
  5. CML (unusual age)
  6. 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:
    • Pancytopaenia
    • 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

  • Major criteria:
    • 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
  • Minor Criteria:
    • Hepatic sinusoidal lymphocytosis
    • Normal cellular and humoral responses to antigenic challenge
    • Hypersplenism
    • 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)
  • Membranous nephropathy:
    • Primary
    • Secondary:
  • Hepatitis B
  • Syphilis
  • SLE
  • Some malignancies
  • Schistosomiasis

References:

  1. Hyper-reactive Malarious Splenomegaly (Tropical Splenomegaly Syndrome); G.G. Crane; Parastitology Today, vol. 2, no. I, 1986
  2. 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
  3. Fakunle Y.Tropical splenomegaly. In: Luzzatto L, ed. Clinics in haematology.London:WB Saunders, 1981: 963–75
Lesson learnt

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.

Facebook