ASH Image Bank (2003); doi:10.1182/ashimagebank-2003-100665
Copyright © 2003 by the American Society of Hematology.
Aplastic Crisis in a Patient with Sickle Cell Disease
John Lazarchick, MD
Medical University of South Carolina
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Figure 1. Numerous sickled RBC's are present (small arrows). A single nucleated RBC is noted (large arrow). Of note is the absence of polychromasia.
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Figure 2. The aspirate is hypocellular consisting primarily of scattered myeloid precursors, small lymphocytes and macrophages. Erythroid precursors are absent.
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Figure 3. The biopsy shows numerous sickled RBCs within the venous sinuses. Plasma cells are prominent around the vascular channels.
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Figure 4. A higher power view of the biopsy illustrates the sinus plugging with sickled RBCs. A single nucleated erythroid precursor is present (arrow).
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Figure 5. A glycophorin immunohistochemical stain for progenitor and mature erythroid cells shows the widespread erythroid stasis within the dilated sinuses. The overall marrow cellularity was 10%, consistent with a diagnosis of moderate to severe hypoplasia.
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Figure 6. A higher power view of Figure 5. shows a single nucleated RBC among the hematopoietic elements present.
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Figure 7. Increased reticulin staining (arrow)is evident concistent with mild to moderate marrow fibrosis.
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Clinical Summary
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37 year old female with history of SS disease, chronic renal failure, nephrotic syndrome and seizure disorder. She was admitted because of increasing fatigue and altered mental status. She had received RBC transfusions in the past and was noted to have multiple RBC antibodies which complicated transfusion therapy on this admission.
T 99 C Pulse 77 BP 134/83 Positive findings on pysical examination were the presence of a 4/6 systolic ejection murmur and diffuse abdominal tenderness. The patient was not easily arousable but no localizing neurologic signs were present.
Laboratory studies: Hemoglobin 1.6g/dL, RBC count 0.54M/cumm, WBC 3900/cumm, platelets 75,000/cumm, absolute reticulocyte count 4000/cumm; hemoglobin electrophoresis S - 90.9%, A2 - 5.3%, F- 3.8%; LDH 466IU/L serum bilirubin 1.1mg/dl; Parvovirus IgG and IgM titers - normal.
Sex
Female
Age
37
Ethnicity
Black
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Diagnosis
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Aplastic crisis in a patient with sickle cell disease
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Discussion
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Aplastic crisis in patients with hereditary hemolytic disorders is usually heralded by a fall in the reticulocyte count. Of note in this case is the corresponding normal serum bilirubin indirect fraction and only minimal increase in LDH. Aplastic crises in these disorders are usually associated with infections, particulary Parvovirus B19 strain, but any infectious agent can be causatory.
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Differential Diagnosis
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Pancytopenia: Aplastic anemia, Myelodysplastic syndrome, Megaloblastic anemia, Marrow replacement (hematopoietic malignancy, fibrosis, metastatic tumor) and Splenomegaly from any cause.
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Copyright © 2003 by the American Society of Hematology.
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