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Bleeding diathesis
8%
13/167
Osteopenia
17%
29/167
Graft-versus-host disease
23%
39/167
Striae distensae
4%
6/167
Thrombosis
35%
59/167
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This patient is a male infant who presents with the triad of eczema, thrombocytopenia, and recurrent bacterial infections consistent with Wiskott-Aldrich syndrome. The only potentially curative treatment for this genetic syndrome is a hematopoietic stem cell transplant, which may be complicated by graft-versus-host disease. Hematopoietic stem cell transplant (HSCT) offers the possibility of a cure in Wiskott-Aldrich syndrome. HSCT involves ablation of the patient’s bone marrow and transfer of matched-donor bone marrow to reconstitute the hematopoietic system. The major complications associated with HSCT include infection and graft-versus-host disease, a syndrome where donor immune cells attack the “foreign” tissues of the transplant recipient. Figure A shows multiple pinpoint areas of blood leakage into the skin (petechiae), which is characteristic of a person with symptomatic thrombocytopenia. Incorrect Answers: Answer 1: A bleeding diathesis does not typically occur in patients who have received a HSCT. The use of HSCT in Wiskott-Aldrich syndrome replaces the hematopoietic progenitor cells which gave rise to the dysfunctional, small platelets seen in the syndrome. Answer 2: Osteopenia, or decreased bone mineral density, is typically asymptomatic but can predispose to bone fractures. It can be seen in patients who are prescribed chronic systemic corticosteroid therapy but is not typically seen in patients who have undergone HSCT. Answer 4: Striae distensae presents with atrophied skin which resembles tissue paper, often in areas of high skin tension. They can be seen on the abdomen during or after pregnancy or in patients with chronic use of potent topical steroids. Striae distensae are not typically seen as a sequela of HSCT. Answer 5: Thrombosis is typically not a sequela of HSCT. Examples of common drugs which increase the rate of thrombotic events include oral contraceptives and anabolic steroids. Bullet Summary: Hematopoietic stem cell transplantation offers the chance of curing Wiskott-Aldrich syndrome but may be complicated by graft-versus-host disease.
3.5
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