Table 2 Biology
Nref | Age yrs | M/F | Hb g/dl | Pl 109/l | WBC 109/l | N 109/l | L 109/l | ANA | DNA | other | Co test | LC | Bone marrow examination |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 (Sarkar et al.2009) | 45 | M | 5.3 | 25 | 2.5 | 1.2 | 1 | 1/640 | + | + | Hypercellular marrow with focal lymphocytic infiltration. | ||
2 (Sacre et al.2009) | 44 | F | 7 | 65 | high | + | - | Hypercellularity with marked reticulin fibrosis. | |||||
3 (Pillai et al.2009) | 40 | F | 10.6 | 25 | 0 | 1/320 | - | + | + | Hypercellular with increased megakaryocytes and reticulin (grade 3). | |||
4 (Aziz et al.2004) | 22 | F | 6.2 | 18 | 1.2 | 1/160 | + | Hypercellularity with an increased number of megakaryocytes. Silver stain showed increased reticulin fibrosis and the presence of patchy areas of collagenized marrow. | |||||
5 (Kiss et al.2000) | 18 | F | 4.2 | 28 | 0.9 | high | aCL | Significant increase in the amount of reticulin fibres (40%, Beumaister 3–4), hypocellularity of the myeloid components and massive lymphocytic infiltration. | |||||
6 (Durupt et al.2000) | 29 | F | 9.4 | 102 | 1.1 | 0.6 | 0.3 | 1/1120 | + | + | All normal hematopoietic elements with a small increase in mature megakaryocytes and marked inflammatory medullar reaction with plasmocytosis, systematized increase in reticulin (grade 1). | ||
7 (Vora et al.1998) | 22 | F | 9 | 60 | 4 | 1/1000 | - | Marked osteomyelosclerosis with severe fibrosis of the marrow spaces and near-total effacement of normal hematopoiesis. | |||||
54 | F | 5.1 | 96 | 3.1 | 2.2 | 0.6 | high | + | aCL, anti-histone, anti-SSA | + | Focal hypercellularity, many reticulin and collagen fibers, normal appearance of the red cell and white cell lines, mild megakaryocytosis with few mildly abnormal megakaryocytes. | ||
9 (Agarwal et al.1995) | 12 | F | 9.6 | 4 | 4.7 | 0.7 | 3.3 | 1/40 | - | - | Extremely hypocellular marrow with residual patches of hematopoietic cells. Reticulin stain showed a marked increase in fine fibrosis, which was diffuse grade 3. | ||
10 (Ramakrishna et al.1995) | 18 | F | 5.4 | 30 | 6 | 1/2560 | + | LA, aCL, antiplatelet | + | + | Markedly hypercellular marrow with erythroid hyperplasia, plentiful megakaryocytes and markedly increased reticulin. | ||
11 (Paquette et al.1994) | 68 | M | 5.7 | 222 | 3.5 | 1/5280 | - | + | Hypercellular, increased stroma and reticulin fibrosis. | ||||
12 (Paquette et al.1994) | 27 | F | 5 | 1 | 5.9 | 1/80 | - | + | - | Fibrosis with megakaryocyte clustering. | |||
13 (Paquette et al.1994) | 23 | F | 9.5 | 55 | 4.2 | high | - | Fibrosis, open sinusoids, megakaryocyte clusters. | |||||
14 (Paquette et al.1994) | 56 | F | 9.7 | 76 | 5.4 | high | + | - | + | Erythroid hyperplasia, increased megakaryocytes, fibroblast proliferation, reticulin fibrosis. | |||
15 (Paquette et al.1994) | 18 | F | 2.7 | 4 | 7.7 | high | + | + | + | Bone marrow fibrosis, hypercellular marrow, increased megakaryocytes with clustering. | |||
16 (Paquette et al.1994) | 70 | F | 4.2 | 20 | 3.8 | high | - | + | + | Advanced bone marrow fibrosis, hypocellular marrow, predominance of megakaryocytes. | |||
17 (Paquette et al.1994) | 62 | F | 9.3 | 35 | 3 | high | - | + | Advanced bone marrow fibrosis, marked osteosclerosis. | ||||
18 (Paquette et al.1994) | 69 | F | 6.8 | 39 | 6.8 | high | - | - | Hypercellular with increased, clustering megakaryocytes; stroma moderately increased by accumulation of fibrillar reticulin. | ||||
19 (Hirose et al.1993) | 54 | F | 6.9 | 17 | 1.7 | 1/320 | - | aCL, LA | + | Marked marrow fibrosis with reduced numbers of erythroid precursors and normal proliferation of both megakaryocytes and myeloid series, the reticulin content was increased. | |||
20 (Borba et al.1993) | 39 | F | 9.7 | 341 | 2.4 | 0.9 | 1/200 | - | anti-neutrophil | + | + | All normal hematopoeitic elements with localised fibrosis, reticulin was moderately increased. | |
21 (Foley-Nolan et al.1992) | 20 | F | 10.5 | 45 | 4.5 | 1.3 | 1.9 | 1/800 | + | + | Hypercellular with a marked increase in the number of megakaryocytes and a diffuse, significant increase in reticulin content. | ||
22 (Inoue et al.1992) | 24 | F | 6.8 | 2 | 3.8 | 1/100 | - | antiplatelet | - | + | Bone marrow fibrosis characterized by marked hyperplasic marrow with an increase in reticulin fibers, all elements including megakaryocytes increased in number. | ||
23 (Matsouka et al.1989) | 60 | F | 20 | 4 | 1.4 | 1.4 | 1/320 | - | - | Presence of dense fibrous tissue with fibroblasts, paucity of immature and polymorphonuclear cells. | |||
24 (el Mouzan et al.1988) | 13 | F | 6.5 | 10 | 1.3 | 0.2 | 1 | 1/640 | + | rhumatoid factor | + | + | Decreased bone marrow activity with 1:1 myeloid to erythroid ratio, megakaryocytes quantitatively normal, increased reticulo-endothelial activity and fibrosis. |
25 (Kaelin and Spivak1986) | 27 | F | 13.1 | 5 | 4.4 | 2.8 | 0.9 | 1/1280 | + | antiplatelet, anti-neutrophil | + | + | Contained all normal hematopoietic elements with a slight increase in mature megakaryocytes, markedly increased reticulin. |
26 (Nanji and Jetha1984) | 28 | M | 8.4 | 1 | 0.3 | high | - | Hypocellular in some areas and hypercellular in others, megakaryocytes decreased in number, increased numbers of histiocytes and fibroblasts, extensive fibrosis and diffuse increased in reticulin. | |||||
27 (Daly and Scott1983) | 16 | F | 7 | 28 | 1.7 | 0.5 | 1/1280 | + | - | + | Marked marrow fibrosis with reduced numbers of erythroid precursors, plentiful megakaryocytes, greatly increased reticulin content. | ||
Present | 29 | F | 2.9 | 15 | 0.48 | 0.28 | 0.08 | 1/1280 | + | anti-SSA, RNP 70, U1-RNP | - | + | Hypercellular marrow (cellularity : 80%) with focal lymphocytic infiltration, dysmyelopoiesis, erythrophagocytosis and grade 1–2 fibrosis. |