Myelodysplasia syndromes during pregnancy
Myelodysplasia syndromes during pregnancy
复制标题
妊娠期骨髓增生异常综合征
DOI：
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发表时间：
1991
影响因子：
3.1
通讯作者：
I. Samaratunga
中科院分区：
文献类型：
--
作者：
A. Pagliuca;G. Mufti;P. Fenaux;C. Silva;I. Samaratunga
To the editor: Pregnancy is usually associated with profound haematological alterations including leucocytosis, slightly reduced platelet counts and an unbalanced increase in red cell and plasma volume leading to anaemia (1). When the full blood count carried out at the first antenatal visit shows a mild cytopenia affecting one lineage alone the distinction between a physiological response to pregnancy and an underlying haematological/medical abnormality may be problematic. When the morphological abnormalities are gross, a diagnosis such as acute leukaemia (2), aplastic anaemia (3) or idiopathic thrombocytopenic purpura (4) may be easy to make. However, when the diagnosis depends on qualitative morphological features as defined in the French-AmericanBritish classification ( 5 ) of the myelodysplastic syndromes (MDS), it may be difficult to make the diagnosis with confidence. This is further compounded by the fact that mild dysplastic features may occur during pregnancy manifested predominantly by dyserythropoiesis. Often, in such situations, additional investigations such as in vitro culture and cytogenetics may be helpful. However, in a proportion of cases there are no definite markers suggesting clonal proliferation. In these cases confirmation of the diagnosis may only be made after reassessment post-partum. This, however, has serious implications for the mother and family who may have to make difficult decisions about termination and treatment, in view of the typically poor long-term survival of MDS. We would like to report our experience of 4 cases of pregnancy-associated MDS and review the literature (Table l). The 1st case, a 28-year-old woman, presented during the 18th week of her second pregnancy with symptoms of anaemia and thrombocytopenia. A diagnosis of refractory anaemia (RA) was made and she was managed symptomatically with blood and platelet transfusions and had a spontaneous vaginal delivery at term. Interestingly the baby was covered with purpura at birth, and was thrombocytopenic with a platelet count of 20 x 109/l. The baby was given a short course of steroids and the platelet count improved over a 3-wk period. The patient had no such response to steroids or high-dose intravenous immunoglobulins and continued to require blood and platelet support. No HLA-compatible sib was available. She had a septicaemic episode 11 months after delivery. Her neutrophil count was now 1.0 x 109/1 and she was commenced on recombinant GM-CSF (Schering-Plough) with a good response in neutrophi1 count. However, arepeat bone marrow 3 months following rGM-CSF therapy continued to show trisomy 8. There was a marked increase in marrow blasts on trephine biopsy and despite discontinuation of GM-CSF she transformed to acute myeloid leukaemia (FAB-M2). She was then started on cytosine arabinoside, daunorubicin and etoposide (ADE) and went into a dysplastic remission with loss of the cytogenetic marker. She had problems with refractory thrombocytopenia requiring HLAmatched platelets. Six weeks later, she relapsed and was again treated with ADE. Following this, she had several haemorrhagic episodes and, despite transfusions of HLA-compatible platelets, she died 23 months after presentation, of pulmonary haemorrhage. The 2nd case, a 30-yr-old woman, presented during the 19th wk of her pregnancy with anaemia and thrombocytopenia. Type 1 blasts accounted for 19% of the myelogram and a diagnosis of refractory anaemia with a excess of blasts (RAEB) was made. An elective Caesarian section was carried out at term with no post-operative bleeding. A healthy female baby was born. Over the ensuing 2 months marrow Type I blasts increased to 25% (RAEB-T) and the patient underwent allogeneic bone marrow transplantation. The patient remains well in haematological and cytogenetic remission. The 3rd case, a 28-yr-old who was initially not aware of being pregnant presented with vasculitis affecting her toes. An initial diagnosis of polyarteritis nodosa was made although a renal biopsy was within normal limits. She was then started on steroids and azathioprine. Her cytopenia progressed and PelgerHuet type neutrophils were noted on peripheral blood smear. Bone marrow examination revealed a hypercellular marrow with obvious trilineage dysplasia. Marrow blasts accounted for 4% of cells at presentation (RA) although this gradually rose to
骨髓增生异常患者的骨髓移植。
影响因子：
39.2
作者：
Appelbaum,FR;Barrall,J;Storb,R;Fisher,LD;Schoch,G;Ramberg,RE;Shulman,H;Anasetti,C;Bearman,SI;Beatty,P
通讯作者：
Beatty,P
使用重组人粒细胞集落刺激因子维持治疗骨髓增生异常综合征患者。
影响因子：
20.3
作者：
Negrin,RS;Haeuber,DH;Nagler,A;Kobayashi,Y;Sklar,J;Donlon,T;Vincent,M;Greenberg,PL
通讯作者：
Greenberg,PL