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Case of the Month - October 2011

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:

Haematology Biochemistry Microbiology/Virology/Immunology
Haemoglobin 4.8g/dl LDH 410 (122-234) Multiple blood cultures negative
MCV 75.8 fl C-reactive protein 118 Sputum AFB & culture negative
RDW 15.5
RPI 0 (later = 2.1)
Urine Prot:Creat 0.20 g/mmol (normal range <0.02) HAV IgM, HBsAg, HCV IgM negative CMV, EBV, Parvovirus, Rubella, HSV all negative
WBC 1.73 x 109/L Albumin 16 g/L Schistosoma serology negative
Neutrophil count 0.81 x 109/L Liver function tests normal CD4 T cell count 113 cells/mm3
Platelets 12 x 109/L Urea and electrolytes normal HIV Viral load unknown
Haptoglobin <0.01 Triglycerides 3.3 ANA negative
Coombs negative    
INR 1.48    
Malaria RDT* negative    
INR 1.48    
Malaria RDT* negative    

* Plasmodium falciparum antigen and pan-malarial antigen

CXR – Normal

Abdominal CT

  • Hepatosplenomegaly
  • Spleen is inhomogenous with multiple peripherally based wedge-shaped hypodensities consistent with infarcts
  • Splenic and portal veins dilated (in keeping with portal hypertension)
  • Horseshoe kidneys with normal excretory function
  • Significant coeliac nodes
  • No free fluid

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:

  • Lung consolidation – no evidence of tuberculosis; possibility of ARDS; extramedullary haematopoiesis
  • Massive hepatosplenomegaly – large amounts of refractile hemosiderin and finer, non-refractile haemozoin pigment (highly suggestive hyperreactive malarial splenomegaly)
  • Significant and severe generalised lymphadenopathy – extramedullary haematopoiesis
  • Membranous nephropathy
  • No evidence of malignancy
  • Question 1: What is the differential diagnosis for massive splenomegaly in this child?

    Continue to Answer 1