Case report:
We report the rare observation about young woman, aged 47 years old, she
was initially referred to our hospital from Central Africa with
complaints of few weeks of epigastralgia with anaemic syndrome and
deterioration of the general state and persistent fever. The initial
physical examination findings revealed fever of 40°C without signs of
infection, pulse 115/min, respiratory rate 20/min, and blood pressure
110/60 mmHg, pallor with frank cutaneous and mucosal jaundice as well as
abdominal distension with hepato-splenomegaly, important edema of the
lower limbs, Vital signs and neurological exam were normal, and no
oxygen was required.
The biological assessment with complete blood count after recent red
cell transfusion revealed pancytopenia (haemoglobin was at 5.8g/dl,
white blood cells counts 2.4 Giga/L, neutrophils level at 1,3 Giga/L,
lymphocytes cells count at 0,9 Giga/L , platelets at 10 Giga/L),
C-Reactive Protein level was at 88mg/L, procalcitonin was negative,
hypoalbuminemia at 24g/l, the research of tuberculosis infections was
done and turned out negative. The rest of the infectious workup has
demonstrated the absence of a toxoplasmosis or leishmaniasis infection,
blood cultures were negatives, the rate of Lactic Acidosis was to
2241UI/L.
The assessment showed also slight cytolysis, an abdominal ultrasound
showed signs of portal hypertension (splenomegaly, dilated portal vein)
and chronic liver disease.
The upper gastroduodenoscopy revealed gastric vascular ectasia, also
known as ‘watermelon stomach’ without associated esogastric varices. To
assess the cause of cirrhosis, the viral serology B and C were performed
and confirmed a co-infection of viral hepatitis B and C . The viral load
results from the quantitative HCV-RNA and HBV-DNA in the serum were
respectively 5,61 log UI/ml (407 380 UI/mL) and 2.02 log UI/ml (104
UI/mL).
Given the persistence of the fever and the worsening of the general
condition of the patient and the negativity of the infectious
assessment, a complementary assessment of febrile pancytopenia was
carried out and made it possible to highlight the presence of images of
hemophagocytosis at the bone marrow aspiration (Figure A), as well as
the existence of hyper-ferritinemia at 1535 ng / ml,
hypertriglyceridemia at 3.9 g / l. Given all these clinical and
biological criteria, the diagnosis retained was the association of a
macrophagic activation syndrome and an HBV-HCV coinfection (codominant)
with compensated cirrhosis.
The patient management combined the treatment for HLHs (Etoposide+
dexamethasone) according to the HLH-94 protocol and antiviral therapy
for HBV-HCV coinfection with supportive care.
This antiviral therapy was based on the European Association for the
Study of the liver (EASL) Guidelines and was adapted to the drugs
available in Morocco: Sofosbuvir + Daclatasvir for 6 months for
hepatitis C and Tenofovir for hepatitis B.
The evolution was favorable with obtaining apyrexia, the cytopenia was
resolved, total disappearance of jaundice, very clear improvement of the
general state, regression of transfusion needs until transfusion
independence, normalization of the biological balance, with undetectable
viral load for both viruses after 3 months of treatment.