Table 2 Clinicopathologic features of five cases of patients with sickle cell disease and bone marrow biopsy

From: Myeloid neoplasms in the setting of sickle cell disease: an intrinsic association with the underlying condition rather than a coincidence; report of 4 cases and review of the literature

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

+

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

  1. Hb hemoglobin, MN myeloid neoplasm, FISH fluorescence in situ hybridization, HU hydroxyurea, N/A not applicable, MGUS monoclonal gammopathy of undetermined significance, MDS myelodysplastic syndrome, PCN plasma cell neoplasm