Case presentation
A 20-year-old non-alcoholic, non-smoker Nepalese man with no significant
past medical history presented to the outpatient department with melena
for 10 days. This was associated with fatigue and generalized weakness
of the body. He denied any abdominal pain, nausea, vomiting, or recent
weight loss. He also gave a history of low-grade fever with an evening
rise of temperature for the same duration. It was not associated with
chest pain, cough, hemoptysis, runny nose, watery eyes, or sore throat.
He had no history of similar illness in the past or any recent sick
contacts. His family history was unremarkable.
Vitals on presentation revealed blood pressure 100/70 mm Hg, pulse
120/min, respiratory rate 24/min, and temperature 100.5 °F. On physical
examination, he was pale and icteric. There was no edema, ecchymosis, or
any palpable lymph nodes. Chest auscultation revealed diminished breath
sounds over the right infrascapular region. The rest of the systemic
examination was normal.
Blood investigations showed: hemoglobin (Hb) 5.3 gm/dl, white blood
cells (WBC) 5400/ mm3 with neutrophils 79% and lymphocytes 18%, and
platelets 319000/ mm3. His renal function tests showed: urea 4.5 mmol/l
and creatinine 95 μmol/L. Liver function test (LFT) showed: total
bilirubin 66 μmol/L, direct bilirubin 7 μmol/L, alanine aminotransferase
(ALT) 23 U/L, aspartate aminotransferase (AST) 38 U/L, and alkaline
phosphatase (ALP) 161 U/L. His lactate dehydrogenase (LDH) was elevated
to 1450 U/L. Direct Coombs test was positive for antibodies against red
blood cells.
Serologies were negative for human immunodeficiency virus (HIV),
hepatitis B virus, and hepatitis C virus. Serological evaluation for
anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid
antibody (ds-DNA), and anti-smooth muscle antibody (ASMA) were negative.
Immunoglobulin levels were normal. A presumptive diagnosis of autoimmune
hemolytic anemia was made, and the patient was started on oral
prednisone 60 mg daily.
Due to the presenting complaint of melena, upper gastrointestinal
endoscopy was done, which revealed a peptic ulcer in the first part of
the duodenum. A tissue sample was taken during endoscopy, which did not
reveal Helicobacter pylori. He was treated with proton pump inhibitors
for his peptic ulcer disease.
Meanwhile, a chest X-Ray showed right-sided pleural effusion (figure 1).
The pleural fluid analysis showed an exudative pleural effusion with
lymphocyte predominance, lymphocyte to neutrophil ratio of 4, and high
adenosine deaminase activity (89 units/L). Computed tomography scan (CT)
of the chest with and without contrast showed multiple centriacinar
nodules giving tree in bud appearance, fibrotic changes in the right
upper lobe, and moderate right-sided pleural effusion suggestive of
tubercular pathology. Acid-fast bacilli (AFB) was not visualized in the
microscopic analysis of the sputum. However, due to high clinical
suspicion, corroborative pleural fluid analysis, and chest imaging
findings, he was started on anti-tubercular treatment with isoniazid,
rifampin, pyrazinamide, and ethambutol.
Follow-up labs showed Hb stable at 5 gm/dl to 7 gm/dl. Platelet count
decreased to 127,000/ mm3 at the time of discharge (figure 2). Following
discharge on anti-tubercular medications and prednisone, he was lost to
follow-up.
The patient presented to our clinic after three months following an
exacerbation of his symptoms after the inadvertent stopping of his
prednisone. He presented with increased fatigue, generalized weakness,
and jaundice. He had no preceding upper respiratory tract symptoms.
Blood investigation this time showed: Hb 4 gm/dl, WBC 6,500/mm3, and
platelets 10,000/ mm3. LFT showed: total bilirubin 48.05 μmol/L and
direct bilirubin 13.51 μmol/L. LDH was elevated to 851 U/L.
The development of thrombocytopenia in a patient with pre-existing
autoimmune hemolytic anemia led us to consider the possibility of Evans
syndrome as the cause of bicytopenia. He was started on intravenous
methylprednisolone 1 gm once daily for three days, followed by oral
prednisone 60 mg daily. However, there was no improvement in his Hb or
platelet count. We considered other immunosuppressive therapies like
intravenous immunoglobulin, rituximab, and cyclosporine as possible
treatment options, but we faced two challenges with their use: fear of
reactivation of TB and the prohibitive cost. Eventually, we decided to
start him on cyclosporine, given its affordable cost despite the risk of
reactivation of TB. He was discharged on oral cyclosporine 10 mg daily
for a month. Unfortunately, he was again lost to follow-up.