Study |
Age, Sex |
Medical
History |
Presenting Symptoms |
ANC |
HGB,PLT |
Bone Marrow |
Treatment |
Outcome |
Levitt LJ |
70 M |
None |
Neutropenic Fever |
48 |
11.3, 28 |
“deficiency of more mature granulocytic forma”, Morphologically recognizable myeloid precursors constitute less than 2% of marrow cellular elements” |
1)Lithium (NR)
2)Prednisone (NR) |
Death attributed to bacteremia as a complication of granulocytopenia |
Barbui T |
63 M |
None |
Isolated neutropenia |
45 |
14, 264 |
“normocellular marrow characterized by almost complete absence of the granulocytic series and a slight increase of lymphocytes and plasma cells” |
1)IVIG (NDR)
2)Antilymphocyte globulin (NR) |
NA |
Ward MS |
15 M |
Beta-thalessemia trait |
Persistant sore-thraot and severe oropharygeal ulceration |
100 |
11.5, 453 |
“virtual absence of granulopoiesis, while the other lines were normal” |
1)IVIG (NDR)
2)CsA (NDR) |
Remained neutropenic but had less infections |
Marmont AM |
56 M |
None |
Recurring bacterial infection |
200 |
NA |
“No granulocytic precusors were found in the BM aspirates” |
1)ALG+CsA+GCSF (NDR)
2)HLA-identical Allogenic BM transplant (NDR)
3)Escalating donar lymphocyte infusion (DR) |
Complete remission of PWCA |
Chakupurakal G |
78 W |
CLL s/p Fludarabine& cyclophosphamide |
Sepsis |
20 |
10.2, 164 |
“Total absence of granulopoiesis” |
1)IVIG (NDR)
2)Azathioprine (NDR)
3)GCSF (NDR)
4)Rituxan (NDR)
5)CsA (DR) |
Neutrophil response at 30 months |
Case # 1PWCA |
67 M |
Waldenstrom Macroglobulinemia |
Recurrent URL |
60 |
9.8, 458 |
“Near absence of granulocytes”, “Paucity of granulocytes in the bone core biopsy and aspirate” |
1)GCSF (NR)
2)Prednisone (NR)
3)Cyclosporine (Dr) |
Neutrophil response at time of writing (~ 11 months) |
Case # 2 PWCA |
23 F |
Developmental Delay |
Recurrent Sepsis |
140 |
12.1, 105 |
“Near absent myelopoiesis”,”Myeloid cells are virtually absent” |
1)GCSF+CsA(DR) |
Neutrophil response at time of writing (~ 8 months) |
ANC: Absolute Neutrophil Count(Cells/uL); Hgb: Hemoglobin (g/dl); Pit: Platelates(k/uL)