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#1 Current Complaint
  • Heme
28yo white pregnant female witha 2 week history of increasing fatigue, easy bruising, fever.  Recent medical history is a bout of RUQ pain 6 months ago, assoc. w/ mild jaundice & fever. Was treated w/ Chloramphenicol for 6 days in another country.  Workup in US showe negative serology for Hep A,B,C.  A diagnosis of non A,B,C hepatitis of unknown etiology was ultimately made
#1 Past Medical History
  • Heme
Patient has vague history of connective tissue disease diagnosed as possible early onset of RA since age 16.  She has been treated with gold salts in the past but is currently stable w/o meds
#1 Family & Social History
  • Heme
Family: non contributory
Social: Works in a leather shoe factory, applies benzene to soften the leather
#1 Laboratory Evaluation
  • Heme
  • Normocytic normochromic anemia
  • Thrombocytopenia
  • Granulocytopenia
  • MCV is upper limits of normal
  • WBC count = 400
  • Lymphocytosis
  • Reticulocyte count low
  • WBC seen= lymphocytes
#1 Physical Exam
  • Heme
mild petechia & some bruising
What elements of the History are helpful in making the diagnosis
  • Heme
28yo white pregnant female with a 2 week history of increasing fatigue, easy bruising, fever. Recent medical history is a bout of RUQ pain 6 months ago, assoc. w/ mild jaundice & fever. Treated w/ Chloramphenicol for 6 days in another country.  Workup in US showed negative serology for Hep A,B,C.  A diagnosis of non A,B,C hepatitis of unknown etiology was ultimately made
PMH: CT disease (possible early onset RA) Gold salt treatment
Social: Works w/ benzene
What info from the Physical exam is helpful?
  • Heme
Mild Petechia: suggests low platelet count (thrombocytopenia)

Bruising
What elements of the Labs are helpful in making a diagnosis?
  • Heme
  • Normocytic, Normochromic anemia
  • Thrombocytopenia
  • Granulocytopenia
  • WBC count 400
  • Relative lymphocytosis
  • Reticulocyte count LOW
Decrased Production!!
Differential Diagnosis
  • Heme (#1)
WBC disorders
  • Myelodysplastic syndromes (PNH, sideroblastic  & chronic monocytic leuk)
  • ALL (bc lymphocytosis)
  • Hairy cell leukemia
What diagnostic test would provide the greatest likelihood of making a definitive diagnosis
  • Heme (#1)
Bone marrow aspirate & Biopsy
  • marrow will show spicules w/ empty fatty space, few hematopoietic cells, lymphocytes, plasma cells, macrophages present
  • Hypo-cellularity & no other causes of pancytopenis are noted
Other tests to rule out other causes of the disease in this case?
  • Heme (#1)
Myelodysplastic syndomes: Cell morphology/cytogenetics, cell surface markers

PNH: + sucrose hemolysis test, abnormal CD59

Hairy cell Leuk & ALL: flow cytometry, cell surface markers & special stains
What is the diagnosis??
  • Heme (#1)
Aplastic Anemia
After diagnosis has been made, what's the best approach to therapy?
  • Aplastic Anemia (#1 Heme)
  • Remove offending agent
  • Blood transfusion support: bc of hypoproduction
  • Bone marrow transplant
  • Immunosuppressants (Cyclosporine, Anti-thymocyte globulin)
  • Corticosteroids
  • Bone Marrow Stimulants
  • Other: tincture of time, experimental drugs
What is the prognosis of Aplastic Anemia (w/ no other health issues)
  • Heme #1
Previously w/o BM transplant & immunosuppression: die w/in 1 year

Now: 75% cure
25% severe GVH disease
15% relapse
25% progress to PNH/MDS/AML
Take home points
  • Heme #1
  • AA: d/o of pluripotent stem cell
  • Dx requires: Pancytopenia, Acellular BM filled w/ fat
  • Do a BM aspirate & Biopsy!
  • Aplastic anemia can progress to PNH, MDS, AML
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