Contributed by S. Chiosea, MD and F. Bontempo, MD
Published on line in March, 2005
A Caucasian woman in her early 40s without significant past medical and family history was found to be thrombocytopenic during the third trimester of her pregnancy. Her platelet count was 24x109/L. She received platelets transfusion and underwent an emergent Cesarean section delivery without complication. Her postpartum course was complicated by a mild hemolytic anemia and she underwent plasmapheresis with no response. She was tapered off of steroids and maintained a platelet count of 45x10^9/L. One year later the patient presented with acute dull pain in right upper abdominal quadrant and enlarged liver. Her LDH was 694 IU/L (<170) and a CT scan of the abdomen revealed hepatosplenomegaly with hepatic (Budd-Chiari syndrome) and splenic veins thromboses.
PREVIOUS MEDICAL HISTORY:
LHD 694 IU/L (<170)
LAP 16 (40-100)
Lupus Anticoagulant Panel
APTT 27.2 seconds (26-36)
PT 13.1 (10.0-12.8)
DRVV 1.0 (0.7-1.1)
TTI 1:50 1.3 (0.9-1.5)
TTI 1:500 1.3 (0.9-1.5)
FVIII 1.88,U/mL (0.5-1.5)
HEX LIP negative
ACA IgG 11.3,GPL (0-23)
ACA IgM 3.5 mpl (0-11)
AT III 107 (80-120)
Folate 28.2 ng/mL (2.6-17.3)
Folate in RBC 817 ng/mL (130-484)
B12 679 pg/mL (211-911)
Ham's test - one of three tests was positive.
Sucrose lysis test - one of three tests was positive.
Bone Marrow, Biopsy
Trilineage hematopoiesis with erythroid predominance and megaloblastoid (occasionally megaloblastic) maturation and with mildly increased reticulin.
Cytogenetic studies No chromosomal abnormalities
Flow Cytometry (FCM)
- 26% of the cells in the monocyte area positive for CD14
- 8% of the cells in the neutrophil area positive for CD16.