E-case #2-B

Ru-Chien Chi, M.D.

June 18-22,2001

Editor's note: Ru just returned from a month of work and travel in Nepal, and wanted to share this case.  She completes her IM training this month and will be entering a geriatrics fellowship.

A 20 yr old previously healthy Nepali male presents with fatigue, cough, hemoptysis, epistaxis, and 6-kg weight loss in 6 mos.  Past medical history is notable for taking OTC medication for "enteric fever."  He's had pesticide exposure from farming.  Denies history of TB, tobacco/alcohol use, and is not married.  Exam is notable for a poorly nourished male without jaundice, lymphadenopathy, or hepatosplenomegaly.  Tachycardia and petechiae are noted.  Laboratory studies revealed WBC 2.3, Hb 5.0, Plt 36 K.

1. What's the differential diagnosis for pancytopenia?

2. What tests would you order?

Differential: "PANCITTO"

PNH

Aplastic anemia

Nutritional (folate, B12 deficiency)

Collagen vascular diseases (SLE, eosinophilic fasciitis)

Infection (TB, EBV, seronegative hepatitis, HIV)

Toxin (Alcohol, benzene, chloramphenicol, gold, penicillamine, allopurinol, NSAIDS, sulfonamides, antithyroid, antiepileptics and antipsychotropics drugs)

Tumor (Myelodysplasia Syndrome, leukemia/lymphoma, myelofibrosis, myelophthisis, thymoma and thymic CA)

Other (Sarcoid, pregnancy, radiation, GVHD)

This patient was diagnosed with aplastic anemia, the most common cause of pancytopenia in young adults. His bone marrow was hypoplastic with less than 25% cellularity.  His exposures included chloramphenicol and insecticides.  Most cases are idiopathic.  The prognosis in aplastic anemia is directly related to the quantitative reduction in peripheral blood cell counts, particularly ANC.  Mortality at two years is 80-90% with patients treated only with blood transfusions and antibiotics.  Bone marrow or peripheral blood stem cell transplant are curative, with survival rates as high as 90%.  Immunosupression  (antithymocyte globulin, cyclosporin) is used in patients who are not candidates for transplant.

References

Brodsky, RA.  Biology and management of acquired severe aplastic anemia.  Current Opin Oncol  Mar 1998; 10(2):95-9.

Guinan, EC.  Clinical aspects of aplstic anemia.  Hematol Oncol Clin N. Am.  Dec 1997; 11(6): 1025-44.

Young, NS.  Acquired aplastic anemia.  JAMA.  July 1999; 282(3): 271-8.