Clinical Images

Pancytopenia due to Peripheral Destruction

Author: Amar H. Kelkar, M.D.

   A 53 year-old male with recent history of pericardial effusion without tamponade presented with fever and night sweats and was admitted for febrile neutropenia. There was no history of immunocompromised health or exposure to pesticides. Physical examination was significant for splenomegaly. The white blood cell count was 1,380 cells/mm³, with the neutrophil count 420 cells/mm³ and atypical lymphocytes were seen on the peripheral blood smear. Serum hemoglobin was 7.1 g/dL with 11,000 platelets/mm³. Evaluation for infection, vitamin deficiencies, hematological malignancies, paroxysmal nocturnal hemoglobinuria, and connective tissue disorders was negative except for isolated ANA titer of 1:320 and positive Cytomegalovirus IgM testing - viral titers were low, however. Bone marrow biopsy would reveal hypercellular marrow (Image 1; 100x) with hypocellular peripheral blood smear (Image 2; 400x). The suspected etiology was acquired peripheral destruction by a recent Cytomegalovirus infection. Hemophagocytic lymphohistiocytosis could not be fully ruled out, though the patient did not meet enough criteria to confirm a diagnosis. Pancytopenia resolved spontaneously after several days.

Multiple Choice Question:

A 25-year-old female presented to the emergency department with persistent fever, generalized weakness, nausea, and decreased appetite associated with 5 kilograms of unintentional weight loss over the past month. There was no significant past medical history. Laboratory findings included pancytopenia with neutropenia. Transaminases were mildly elevated without hyperbilirubinemia. A coagulation panel reveals International Normalized Ratio (INR) 1.3, Partial Thromboplastin Time (PTT) 40 seconds, and Fibrinogen 120 mg/dL. Serum ferritin level was 40,421 ng/mL. Physical examination revealed notable splenomegaly with tenderness of the left upper quadrant and mild diffuse tender cervical lymphadenopathy. Which of the following is most likely to confirm the diagnosis?

1. Serum Protein Electrophoresis

2. Bone Marrow Biopsy

3. Osmotic Fragility Testing

4. Antinuclear Antibody (ANA) titer with Extractable Nuclear Antigen (ENA) panel

5. Viral Studies including Cytomegalovirus (CMV) and Epstein-Barr virus (EBV) titers

Click here for answer

References:

1. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. The Lancet. 2014 Apr 26; 383: 1503-1516. doi: 10.1016/s0140-6736(13)61048-x.

Author Bio:

Amar Kelkar is a PGY-3 Internal Medicine Resident at the University of Illinois College of Medicine at Peoria. He is currently applying to fellowship programs in Hematology/Oncology. Current research interests include acquired factor inhibitors and viral-host interactions related to bleeding, thrombosis, and malignancy. Other interests include teaching, advocacy, and humanism in medicine.