A 45-year-old male received an allogeneic bone marrow transplant for acute lymphatic leukaemia (ALL). Twenty-six days after the transplant he developed severe gastroenteritis and a maculopapular skin rash and respiratory insufficiency.
The following investigations were performed:
Test |
Value |
Normal Adult Range |
Haemoglobin* |
94G/L |
110 – 150 |
WCC* |
2.3 x 109/L |
4.0–10.0 |
Platelets* |
54 x 109/L |
150 – 300 |
Sodium* |
132 mmol/L |
135 – 145 |
Potassium* |
3.4 mmol/L |
3.5–5.0 |
Urea* |
8.2 mmol/L |
4.0–6.0 |
Creatinine |
100 µmol/L |
40 – 120 |
Bilirubin* |
67 µmol/L |
<25 |
ALP* |
265 IU/L |
<125 |
AST |
40IU/L |
<40 |
ALT* |
51IU/L |
<40 |
Coagulation profile: Normal
Stool:
a) List three possible diagnoses.
Over the next 3 weeks, he developed generalized oedema predominantly in the trunk and lower extremities.
An ultrasound Doppler study of the abdomen revealed dilated portal vein and inferior vena cava and the following pressure measurements were obtained:
Test |
Value |
Normal Adult Range |
Portal pressure* |
18 mmHg |
8– 10 |
Infrahepatic IVC* |
20 mmHg |
9– 11 |
Hepatic vein* |
8 mmHg |
9– 10 |
Suprahepatic IVC |
8 mmHg |
7– 8 |
Right atrium |
6 mmHg |
5- 10 |
a)
Sepsis
CMV infection
Graft v Host disease
b)
Veno-occlusive disease of the liver
c)
TIPS procedure
Diuretics
Fluid restriction
Somewhat unfairly, the college has presented us with a patient suffering from some nonspecific symptoms. This young man is anaemic, thrombocytopenic, and has raised LFTs which may explain the high bilirubin. On the plus side, he seems to have engrafted.
Differentials would have to include GVHD (given the rash), CMV infection (given the gastroenteritis) and maybe sepsis - but DIC is ruled out by the absence of coagulopathy.
Generally speaking, the manifestatations of GVHD are as follows:
The college reminds us to think broad by throwing a sepsis and a CMV in there. Manifestations of CMV infection following bone marrow transplant are protean, and may include all of the features mentioned in the college question.
Is there an organ system it does not affect?
The suddenly increased portal pressure (normal is 5-10mmHg) is suggestive of veno-occlusive disease of the bone marrow transplant recipient. These pressure measurements are not diagnostic values, however. There are the Seattle criteria and there are the Baltimore criteria, neither of which actually mention any pressures at all - they demand hepatomegaly, ascites and raised bilirubin.
However, the pressure values are given for a reason.
The current BCSH/BSBMT guidelines suggest the following management options:
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