Table 2 Clinicopathologic features of five cases of patients with sickle cell disease and bone marrow biopsy
Item | Case 5 | Case 6 | Case 7 | Case 8 | Case 9 |
|---|---|---|---|---|---|
Sex | F | F | M | F | F |
Age (year) | 9 | 9 | 22 | 31 | 61 |
Hemoglobin type | SC | SS | Sβ+ | SS | SC |
Clinical history | Retinopathy, splenomegaly | Sickling crisis, jaundice, splenomegaly | Hepatitis, Splenomegaly | Sickling crisis, parvovirus B19 + , transfusion reaction | Chronic anemia |
Presentation at bone marrow biopsy | Neutropenia | Neutrophilia | Anemia, fever | Leukocytosis | MGUS |
Treatment for HbSS or equivalent Hb | Supportive care | Supportive care | Supportive care | Supportive, HU (1000 mg/day) | Supportive care |
CBC (WBC/Hb/MCV/PLT) | 2.4/9.8/76/118 | 40.8/7.5/83/402 | 8.4/5.8/77/293 | 8.6/5.2/63/723 | 8.6/9.5/79/221 |
Blood smear findings other than RBC morphology | Neutropenia, no blasts | Left-shifted neutrophilia, toxic granules | No blasts | No smear available | No smear available |
Marrow cellularity | 60% | 100% | 90% | 20% | 60% |
Dysplasia | No | No | No | No | No |
Marrow blasts (%) | 1 | 0 | 2 | 1 | 2 |
Immunophenotype | Negative | Negative | Negative | Negative | Negative |
TP53 stain | Negative | Negative | Negative | Negative | Negative |
Karyotype | 45,XX [20] | 46,XX,inv(9)(p11q13)c[20] | 46,XY[20] | 46,XX,t(7;11)(q22;q12)c[20] | 46,XX[20] |
Interphase FISH | Negative for MDS panel | Negative for BCR/ABL | Negative for MDS panel | Negative for MDS panel | Negative for myeloma panel |
Diagnosis | No MN | No MN | No MN | No MN | No MN/PCN |
Follow-up (mo) | 54 | 36 | 2 | 24 | 35 |
Outcome | No MN | No MN | No MN | No MN | No MN |