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)
NR: (Non-response) No neutrophil response treatment; NDR: (Non durable response) Neutrohil response with subsequent relaps; DR: (durable response) Neutrohil response without subsequent relapse: NA: Not avialable or uninterpretable; CLL Chronic lymphocytic leukemia; URL: Upper respiratiory infection; PWCA : Pure White cell aplasia; csA: Cyclosporine; IVIG : Intravenous immunoglobulin; ALG: Antilymphocyte globulin; GCSF: Granulocyte colony-stimulating factor
Table 1: Review of literature of PWCA not considered to be caused by drugs, thymomas, infections, or autoimmune diseases.