A Case of Typhoid Fever with Myelodysplastic Syndrome Resulting in Severe Pancytopenia

INTRODUCTION A patient diagnosed with immunoglobulin-M-positive typhoid fever presented with myelodysplastic syndrome. Bone marrow aspirate was suggestive of dysgranulopoiesis in the form of hypo-lobulated neutrophil, abnormally segmented neutrophil and hypercellular for age. This case report highlights a rare presentation where myelodysplastic syndrome has coexisted with typhoid fever resulting in severe pancytopenia. CASE REPORT A 68-year-old female presented with complaints of altered mental sensorium for 1 day, shortness of breath for 7 days, fever for 10–15 days, generalised body swelling and easy fatiguability for 1½ months. In the past 1 day, she developed hypoactive delirium with reduced verbal response and disorientation with worsening of shortness of breath. At presentation, Glasgow Coma Scale (GCS) = E3V1M5, temperature = 101°F, pulse = 114/min, blood pressure = 86/50 mmHg and urine output was 50–100 mL/h. A complete hemogram showed haemoglobin (Hb) – 1.6 g/dL, total leucocyte count – 900, absolute neutrophil count – 540, platelet count – 8000, total bilirubin/direct bilirubin – 2.80/0.67, serum glutamic oxaloacetic transaminase/serum glutamate pyruvate transaminase – 24/12, total protein/albumin – 6.10/3.60, blood urea/serum creatinine – 32.6/0.58 and serum sodium/potassium – 145/3.91. Arterial blood gas (ABG) analysis showed pH – 7.281, HCO3 – 7.9, PaCO2 – 15.6 and lactate – 15.8, suggestive of severe metabolic acidosis. The patient was intubated because of low GCS and septic shock. Three units of packed red blood cells (RBC) were transfused in view of severe anaemia. Three units of random donor platelets were also transfused. Subsequent ABG showed pH – 7.486, HCO3 – 20.1, PaCO2 – 26.3 and lactate – 7.0. Once stable, the patient was evaluated for causes of pancytopenia with altered mental sensorium. Fever workup came negative except for Typhidot IgM, which came positive. Procalcitonin was 1.32 ng/mL. For altered sensorium, a neurology opinion was taken. Lumber puncture and computed tomography scan were done, but reports came out to be normal. Peripheral blood smear showed normocytic normochromic RBCs with anisocytosis with leucopenia with neutrophilic preponderance. Indirect and direct agglutination tests subsequently came negative, with a reticulocyte count of 0.43%. Empirical antibiotic treatment was started for typhoid, including injection levofloxacin 750 mg intravenous (IV) and injection ceftriaxone 2 g IV per day. Bone marrow biopsy was done, and investigations, including bone marrow aspiration slides, cytogenetics and bone marrow culture, were sent. Provisional bone marrow aspirate was suggestive of myelodysplastic syndrome with dysgranulopoiesis in the form of hypo-lobulated neutrophil, abnormally segmented neutrophil and hypercellular for age. The patient gradually improved over the next 2–3 days. The fever subsided, sensorium improved and white blood cell counts, Hb and platelet count showed an incremental trend. Gradually, ventilatory support was reduced to Continuous positive airway pressure/pressure support (CPAP/PS). The patient was subsequently extubated. The patient was maintaining a saturation of 95% with oxygen through nasal prongs at 2 L/min. The patient was gradually shifted to room air as her general condition improved. DISCUSSION Typhoid or enteric fever is an abdominal infection commonly caused by Salmonella enterica serotype Typhi (formerly Salmonella typhi). Other organisms responsible for enteric fever include Salmonella paratyphi and non-typhoidal Salmonellae (i.e., Salmonella enteritidis and Salmonella typhimurium). Commonly caused by ingestion of contaminated water or food, it results in fever, chills and abdominal pain. Diarrhoea (more common) and constipation are gastrointestinal manifestations, with splenomegaly and intestinal perforation (commonly ileum) seen in severe/chronic cases. 'Typhoid encephalopathy', characterised by altered consciousness, delirium and confusion, is seen in 17% of patients with typhoid.[1] Abnormal liver function tests are also observed.[2] Haematological findings include leucopenia with neutropenia. Severe pancytopenia with marrow aplasia and hemophagocytosis although rare has also been reported with typhoid fever.[3] For pancytopenia evaluation, a bone marrow biopsy was done, which showed features of myelodysplastic syndrome. A myelodysplastic syndrome is a heterogeneous group of myeloid neoplasms resulting in haemopoietic stem cell dysplasia and ineffective haematopoiesis.[4,5] Clinically, it presents as a decrease in the number of RBCs, white blood cells and platelets. Most commonly presenting in old age, i.e., above 65 years, it can occur de novo or can be attributed to toxins or chemotherapeutic agents.[5] Our patient did not have a history of any of these. The prognosis of this condition is variable and depends on the severity of pancytopenia and karyotype analysis. Our main aim in reporting this case is to highlight the rare finding of typhoid IgM positive with myelodysplastic syndrome. One should not only rule out typhoid fever in patients having fever with pancytopenia but also go for an additional workup to determine if any other associated abnormality is complicating the clinical course. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.