Clinical and laboratory data of the patients studied
Patient no. . | Sex . | Age (y) . | Disease* . | Duration (mo) . | Previous medications . | Antibody specificity† . | Bone marrow‡ . | Peripheral blood counts1-153 . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Cellularity . | Hb (g/dL) . | WBC (×109/L) . | Neutro (×109/L) . | Lympho (×109/L) . | Plt (×109/L) . | Ret (×109/L) . | |||||||
1 | F | 52 | Idiopathic AIN | 60 | G-CSF | ND | Increased/hypercellular myeloid series with normal maturation | 12.3 | 1.2 | 0.5 | 0.3 | 165 | 46 |
2 | F | 58 | AIN/Sjogren | 12 | None | ND | Normal/normal myeloid series | 10.8 | 1.9 | 0.8 | 0.8 | 152 | 45 |
3 | F | 20 | AIN/thyroiditis | 15 | G-CSF | anti-NA1 | Normal/normal myeloid series | 13.8 | 2.0 | 0.2 | 1.2 | 250 | 55 |
4 | F | 25 | Idiopathic AIN | 7 | None | anti-NA1 | Increased/hypercellular myeloid series with left shift | 10.5 | 2.3 | 0.7 | 1.4 | 154 | 45 |
5 | F | 19 | Idiopathic AIN/ pancytopenia | 66 | PDN/γ-globulin | anti-NA1 | Increased/hypercellular myeloid series with left shift, increased Meg series | 8.9 | 3.6 | 0.7 | 1.7 | 140 | 55 |
anti-GPV | |||||||||||||
6 | M | 35 | Idiopathic AIHA | 8 | PDN/γ-globulin | Markedly increased/hypercellular erythroid and Meg series | 12.9 | 6.1 | 3.3 | 2.2 | 360 | 114 | |
7 | M | 46 | ITP/AIHA | 48 | PDN/AZA | anti-GPIIb/IIIa | Mildly increased/hyperplasia of Meg series | 12.0 | 7.5 | 4.5 | 1.6 | 63 | 65 |
γ-globulin | anti-GPIb/IX | ||||||||||||
8 | F | 30 | ITP | 3 | None | anti-GPIIb/IIIa | Mildly increased/hyperplasia of Meg series | 16.0 | 5.0 | 2.4 | 1.9 | 94 | 50 |
9 | F | 24 | ITP | 28 | PDN/AZA/CSA | anti-GPIb/IX | Mildly increased/hyperplasia of Meg series | 12.0 | 2.7 | 1.9 | 0.4 | 87 | 60 |
VCR/CAMPATH-1 | |||||||||||||
γ-globulin | |||||||||||||
10 | F | 20 | ITP/AIHA/Behcet disease | 84 | PDN | ND | Normal/slightly increased Meg series | 12.1 | 4.4 | 2.3 | 1.7 | 173 | 55 |
11 | M | 65 | Primary PRCA1-155 | 48 | PDN/AZA/CSA | Normal/near absence of erythroid series, normal myeloid and Meg series | 6.1 | 4.6 | 1.7 | 1.6 | 110 | 35 | |
12 | F | 48 | Primary PRCA1-155 | 24 | PDN/ALG/CSA | Normal/reduced erythroid series, normal myeloid and Meg series | 11.6 | 5.6 | 3.4 | 1.8 | 255 | 18 | |
13 | M | 30 | Primary PRCA1-155 | 36 | PDN/CSA | Normal/near absence of erythroid series, normal myeloid and Meg series | 8.9 | 4.3 | 2.5 | 1.7 | 333 | 20 | |
14 | F | 34 | Primary PRCA | 24 | PDN/CSA | Normal/absence of erythroid series, normal myeloid and Meg series | 5.3 | 5.7 | 3.5 | 2.0 | 205 | 25 | |
15 | F | 34 | Primary PRCA | 14 | PDN/CSA | Normal/reduced erythroid series, normal myeloid and Meg series | 8.4 | 5.9 | 3.7 | 1.9 | 335 | 25 |
Patient no. . | Sex . | Age (y) . | Disease* . | Duration (mo) . | Previous medications . | Antibody specificity† . | Bone marrow‡ . | Peripheral blood counts1-153 . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Cellularity . | Hb (g/dL) . | WBC (×109/L) . | Neutro (×109/L) . | Lympho (×109/L) . | Plt (×109/L) . | Ret (×109/L) . | |||||||
1 | F | 52 | Idiopathic AIN | 60 | G-CSF | ND | Increased/hypercellular myeloid series with normal maturation | 12.3 | 1.2 | 0.5 | 0.3 | 165 | 46 |
2 | F | 58 | AIN/Sjogren | 12 | None | ND | Normal/normal myeloid series | 10.8 | 1.9 | 0.8 | 0.8 | 152 | 45 |
3 | F | 20 | AIN/thyroiditis | 15 | G-CSF | anti-NA1 | Normal/normal myeloid series | 13.8 | 2.0 | 0.2 | 1.2 | 250 | 55 |
4 | F | 25 | Idiopathic AIN | 7 | None | anti-NA1 | Increased/hypercellular myeloid series with left shift | 10.5 | 2.3 | 0.7 | 1.4 | 154 | 45 |
5 | F | 19 | Idiopathic AIN/ pancytopenia | 66 | PDN/γ-globulin | anti-NA1 | Increased/hypercellular myeloid series with left shift, increased Meg series | 8.9 | 3.6 | 0.7 | 1.7 | 140 | 55 |
anti-GPV | |||||||||||||
6 | M | 35 | Idiopathic AIHA | 8 | PDN/γ-globulin | Markedly increased/hypercellular erythroid and Meg series | 12.9 | 6.1 | 3.3 | 2.2 | 360 | 114 | |
7 | M | 46 | ITP/AIHA | 48 | PDN/AZA | anti-GPIIb/IIIa | Mildly increased/hyperplasia of Meg series | 12.0 | 7.5 | 4.5 | 1.6 | 63 | 65 |
γ-globulin | anti-GPIb/IX | ||||||||||||
8 | F | 30 | ITP | 3 | None | anti-GPIIb/IIIa | Mildly increased/hyperplasia of Meg series | 16.0 | 5.0 | 2.4 | 1.9 | 94 | 50 |
9 | F | 24 | ITP | 28 | PDN/AZA/CSA | anti-GPIb/IX | Mildly increased/hyperplasia of Meg series | 12.0 | 2.7 | 1.9 | 0.4 | 87 | 60 |
VCR/CAMPATH-1 | |||||||||||||
γ-globulin | |||||||||||||
10 | F | 20 | ITP/AIHA/Behcet disease | 84 | PDN | ND | Normal/slightly increased Meg series | 12.1 | 4.4 | 2.3 | 1.7 | 173 | 55 |
11 | M | 65 | Primary PRCA1-155 | 48 | PDN/AZA/CSA | Normal/near absence of erythroid series, normal myeloid and Meg series | 6.1 | 4.6 | 1.7 | 1.6 | 110 | 35 | |
12 | F | 48 | Primary PRCA1-155 | 24 | PDN/ALG/CSA | Normal/reduced erythroid series, normal myeloid and Meg series | 11.6 | 5.6 | 3.4 | 1.8 | 255 | 18 | |
13 | M | 30 | Primary PRCA1-155 | 36 | PDN/CSA | Normal/near absence of erythroid series, normal myeloid and Meg series | 8.9 | 4.3 | 2.5 | 1.7 | 333 | 20 | |
14 | F | 34 | Primary PRCA | 24 | PDN/CSA | Normal/absence of erythroid series, normal myeloid and Meg series | 5.3 | 5.7 | 3.5 | 2.0 | 205 | 25 | |
15 | F | 34 | Primary PRCA | 14 | PDN/CSA | Normal/reduced erythroid series, normal myeloid and Meg series | 8.4 | 5.9 | 3.7 | 1.9 | 335 | 25 |
Hgb indicates hemoglobin; WBC, white blood cells; Neutro, neutrophils; Lympho, lymphocytes; Plt, platelets; Ret, reticulocytes; AIN, autoimmune neutropenia; AIHA, autoimmune hemolytic anaemia; ITP, autoimmune thrombocytopenic purpura; PRCA, pure red cell aplasia; Meg series, megakaryocytic series; G-CSF, granulocyte-colony stimulating factor; PDN, prednisone; AZA, azathioprine; CSA, cyclosporin A; VCR, vincristine; ALG, antilymphocyte globulin; ND, not defined; NA1, neutrophil antigen 1; GP, platelet glycoprotein.
AIN diagnosis was based on the presence of specific antineutrophil antibodies in patient serum detected with chemilumonescence and immunofluorescence techniques.23 ITP diagnosis was based on the presence of antiplatelet antigens or anti-GP specific antibodies in patient sera and platelets detected by means of flow cytometry (platelet flow cytometric immunofluorescence technique; PIFT) and ELISA (monoclonal antibody immobilization of platelet antigen assay; MAIPA).24
In cases of AIN and ITP.
At diagnosis.
Performed at the time of study.
Bone marrow T-cell receptor and immunoglobulin gene rearrangement studies had been performed to exclude clonality.