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.