Woman with Abdominal Pain and Thrombocytopenia

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Ji Lu

A 42-year-old African-American woman developed dull crampy abdominal pain without accompanying nausea, fever, or diarrhea. Pain persisted over the next 4 days, and she noticed blurred vision and two bruises on her legs, although she did not recall ever falling or bumping into anything. When her symptoms did not improve, her sister brought her to the hospital. The patient worked as a nurse's aide, took no prescribed or over-the-counter medications, and did not acknowledge consumption of alcohol or illicit drugs. She was not sexually active.

On physical exam she was normotensive and afebrile; there were several 4-7-cm ecchymoses on the right knee and left tibia; neurological exam was nonfocal; her abdomen was mildly tender without palpable liver, spleen, or mass. The remainder of her exam was normal. Initial laboratory findings were as follows:

Reference

Value,

Interval,

Reference

Conventional

Conventional

Value,

Interval,

Analyte

Units

Units

SI Units

SI Units

WBC

17.6 x 103/mL

4-10

17.6 x 109/L

4-10

Hemoglobin

5.0 g/dL

12-15

50 g/L

120-150

Hematocrit

19%

36-48%

0.19 volume

0.36-0.48

MCV

61 fL

80-98

Same

Reticulocyte %

8.2%

0.5-1.5%

0.082 fraction

0.005-0.015

Platelet count

17 x 103/mL

140-400

17 x 109/L

140-400

Prothrombin time

14 s

11-15

Same

aPTT

26.45

23-36

Same

Fibrinogen

454 mg/dL

140-400

4.54 g/L

1.4 - 4.0

Billirubin, total

3 mg/dL

0.3-1.2

51.3 mmol/L

5.1-20.5

Bilirubin, indirect

2.5 mg/dL

<0.2

42.8 mmol/L

< 3.42

Lactate dehydrogenase

4500 U/L

50-150 U/L

76.5 mkat/L

2.50-4.17

Creatinine

1.4 mg/dL

0.6-1.1

124 mmol/L

53-97

Haptoglobin

<7 mg/dL

35-164

<7 mg/L

350-1640

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Figure 66.1 100x magnification of peripheral blood smear showing schistocytes (black arrows) and polychromatophilic red cells (white arrows).

A review of the patient's peripheral blood smear confirmed the low platelet count and elevated WBC consisting of neutrophils with immature forms limited to bands and metamyelocytes (left shift). Red cell morphology was notable for severe poikilocytosis (variation in shape) and anisocytosis (variation in size) dominated by many schistocytes (small, irregular, fragmented red cells) and polychromatophilic red cells (larger, blue-gray cells, consistent with increased reticulocyte count) (Fig. 66.1). The history, laboratory studies, and peripheral blood smear findings were consistent with microangiopathic hemo-lytic anemia due to thrombotic thrombocytopenia purpura (TTP). She was infused with four units of fresh-frozen plasma and two units of red cells while waiting for a central venous catheter to be inserted for plasma exchange.

Daily plasma exchange was initiated and abdominal pain resolved within several days, but platelet count (range 18-62 x 103/mL) and LDH did not normalize after 15 days despite addition of prednisone and replacement with cryoprecipitate-poor plasma. The patient tolerated a laproscopic splenectomy on day 16 without serious bleeding complications, and daily plasma exchange was continued, but her platelet count did not improve until rituximab was started. She was discharged on day 25 with a platelet count of 333 x 103/mL, and has remained in clinical remission for 38 months.

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