Dr Gary Reubenson and Dr Gillian Sorour, Dept of Paediatrics and Child Health, University of Witwatersrand
A 13 year old girl was referred from a district hospital to an academic secondary level hospital. She had been born in South Africa, but had lived in Mozambique continuously for the last 5 years. She had recently returned to South Africa as she was unwell and her mother lived here.
According to the referral note, she had been ill for 14 days with high fevers and chills, headaches and a distended abdomen. The doctors at the referring hospital had also identified pancytopenia.
She had apparently been previously well with no prior hospital admissions. Initial investigations included two reactive Rapid-HIV tests, a negative falciparum malaria antigen test and a negative malaria smear.
On examination, she was ill-looking, her weight plotted on the 3rd percentile and her height on the 10th percentile. Her temperature was 40°C, she had marked pallor and generalized significant cervical, axillary and inguinal lymphadenopathy. She also had bilateral pedal and peri-orbital oedema.
On abdominal examination her liver was palpable 5cm and her spleen 18cm below the costal margin. There was no ascites or prominent abdominal wall veins. Her initial investigations showed:
* Plasmodium falciparum antigen and pan-malarial antigen
CXR – Normal
Progress in the ward:
Spiked high temperatures (up to 40 °C) throughout her admission
Started on intravenous quinine, but stopped after 2 days
7 days ceftriaxone
Transfused packed cells repeatedly
Started on empiric 4-drug TB treatment
ARVs started 12 days after TB treatment initiated
Cotrimoxazole changed to dapsone in view of persistent anemia
She underwent a bone marrow examination:
The aspirate showed a hypercellular marrow with hemopoietic activity in all 3 cell lines. The trephine was of sub-optimal quality, but confirmed hypercellularity. There was some evidence of non-refractile pigment noted in macrophages and an abnormal infiltrate could not be excluded.
The conclusion of the test was that her pancytopenia was probably multifactorial due to a combination of dyshaemopoiesis (infection and/or nutritional), reticul-endothelial iron blockade, DIC and hypersplenism. The child was sent to a paediatric haematology-oncology subspecialty unit for biopsy and unfortunately demised unexpectedly the night before the scheduled procedure.
A postmortem was performed:
Question 1: What is the differential diagnosis for massive splenomegaly in this child?
Answer to Q1
Question 2: What additional blood test(s) would have confirmed hyperreactive malarial splenomegaly?
Answer to Q2
Question 3: What are the diagnostic criteria for hyperreactive malarial splenomegaly?
Answer to Q3
Question 4: What are the possible causes for her nephrotic syndrome?
Answer to Q4
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.
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