伴血细胞减少系统性红斑狼疮34例骨髓细胞形态学分析

目的 了解伴血细胞减少的系统性红斑狼疮(SLE)骨髓细胞形态学变化.方法 对34例外周血细胞减少的SLE患者(WBC<4×109/L, 或Hb<100 g/L, 或Pt<100×109/L)的骨髓涂片进行回顾性细胞形态学检查,并与26例特发性血小板减少性紫癜(ITP)、18例骨髓增生异常综合征的难治性贫血(MDS-RA)患者以及14例健康对照者综合分析.结果 34例SLE患者中24例(70.6%)骨髓发育不良,包括红系多核(三核或更多)10例(29.4%),巨幼样变8例(23.5%), Pelger-Hüet样畸形12例(35.3%),多核巨核细胞8例(23.5%),小型巨核细胞10例(29.4%). 结论在伴血细胞减少的SLE患者中能观察到骨髓多系细胞发育不良,这些改变说明骨髓可能是SLE侵犯的靶器官之一.     

系统性红斑狼疮患者血液学指标相关性43例分析

目的研究系统性红斑狼疮(SLE)患者的血象及骨髓特点.方法对43例SLE患者的血象及骨髓象进行涂片染色镜检并进行临床观察.结果SLE临床表现以贫血为主(86%),另有不同程度的发热(占53.5%),面部红斑(占65.1%),部分患者并发有其他症状.SLE患者血象WBC、Hb及PLT均有不同程度减少;骨髓象增生活跃程度不一,部分患者可出现原始细胞、早幼粒细胞及嗜酸性细胞增多.红系80%以上增生良好,但也有部分患者表现为缺铁性贫血、溶血性贫血及混合性贫血.巨核细胞增生正常的占65.1%,另有少数增生减低或缺如.结论SLE临床表现不一,多以贫血、血小板减少等为首发症状,部分患者骨髓象可出现原始细胞及早幼粒细胞增多.     

原发性干燥综合征103例血液学变化

目的 了解原发性干燥综合症（pSS)的血液学变化。方法 对103例pSS中患者外周血、骨髓象治疗前后情况进行总结分析。结果 pSS合并多种血液学异常,其中贫血最常见,占51%,单纯贫血占34%。白细胞减少占25%,血小板减少占23%,全血细胞减少占7.7%。经皮质激素治疗后,外周血及骨髓象均有不同程度的改善。结论 pSS的血液系统异常与免疫因素有关。     

血小板减少症患者1400例病因分析

目的探讨初诊血小板减少症(thrombopenia,TP)住院患者的病因和特点。方法收集2009年10月至2010年11月1 400例住院初诊TP患者的病历资料,回顾性分析病因构成、血小板参数变化、实验室指标变化和骨髓特点。结果①病因构成比:非血液系统疾病占57.9%,血液系统疾病占29.6%,诊断不明12.0%,假性血小板减少症0.5%。②非血液系统疾病引起TP的患者血小板数减少程度较血液系统疾病患者轻,分别为68(3～99)×109/L和27(1～98)×109/L,差异有统计学意义(P<0.05)。③巨幼细胞性贫血(MA)所致TP患者的血小板分布宽度(PDW)升高,实体肿瘤和非霍奇金淋巴瘤所致TP患者的PDW升高或正常。再生障碍性贫血和骨髓增生异常综合征所致TP患者的平均血小板体积(MPV)降低或正常,MA和急性冠脉综合征所致TP患者的MPV升高或正常。④感染和药物所致TP患者的乳酸脱氢酶升高或正常,均与血小板计数呈负相关。肾功能不全所致TP患者的血小板计数与肌酐水平呈正相关。⑤459例患者行骨髓检查,其中血液系统疾病309例、诊断不明20例、非血液系统疾病130例,骨髓为增生活跃以上110例、增生低下20例,巨核细胞总数大于150个15例、小于7个24例。结论 TP病因复杂,非血液系统疾病为主要原因,其血小板减少多为Ⅰ～Ⅱ度,骨髓检查多无特征性改变,需结合病史、症状、体征及理化检查综合分析寻找病因。     

血小板生成减少引起的血小板减少症

纯巨核细胞生成障碍引起的血小板减少是一种少见的疾病,其特征是骨髓巨核细胞明显减少或完全缺乏导致血小板减少,而其他血细胞成分很少累及。巨核细胞生成障碍性血小板减少患者伴有其他细胞系轻度异常,大红细胞或红细胞生成障碍较少见,这些异常可能是骨髓增生异常或再生障碍性贫血(下称再障)的一个前驱表现。获得性巨核细胞生成障碍可能与以下因素有关,如某些病毒如微小病毒、人类免疫缺陷病毒(HIV-1)等感染;某些药物如噻嗪类利尿剂、雌激素、     

165例系统性红斑狼疮患者血液系统受累情况分析

目的 探讨系统性红斑狼疮(SLE)患者血液系统受累特点,为其诊治提供依据.方法 对165例确诊的SLE患者血液系统受累情况进行回顾性分析.结果 本组96例有贫血,其中慢性疾病性贫血(ACD) 63例,自身免疫性溶血性贫血(AIHA) 11例;72例外周血白细胞下降,65例血小板下降.49例行骨髓检查,以增生活跃为主(44例).结论 SLE血液系统受累发生率高、范围广.对血象异常的育龄女性患者应注意进行SLE的相关筛查.对于诊断为AIHA或特发性血小板减少性紫癜的患者,应警惕同时存在SLE的可能性.     

难治性贫血伴环形铁粒幼细胞患者SF_3B_1基因突变病例报告

目的:提高对难治性贫血伴环形铁粒幼细胞(RARS)患者检测SF_3B_1基因突变的认识。方法:回顾性分析2例RARS并SF_3B_1(K700E)基因突变患者的临床资料并进行相关文献复习。结果:2例患者均有头昏、乏力症状,均为难治性贫血,白细胞计数和分类正常,血小板计数增高,骨髓象粒系减低,红系增生占56%~72.4%,其中畸形红细胞占12%~14%,粒系、巨核细胞也见畸变。细胞外铁~,铁粒幼细胞74%~95%,其中环形铁粒幼细胞占24%~28%。骨髓活检增生明显~极度活跃(占80%),染色体核型正常,JAK_2基因阴性,SF_3B_1(K700E)基因阳性,2例均诊断RARS(SF3B1K700E基因突变)。文献报告RARS伴血小板增多又伴JAK_2基因突变,患者用小剂量雷那度胺治疗有效。结论:对RARS患者进行SF_3B_1、JAK_2等基因检测,可给患者提供适当的治疗方法,做好针对个体化精准治疗,改善贫血,提高疗效。     

慢性再生障碍性贫血转化为骨髓增生异常综合征2例

例 1 女性 ,49岁。面色苍白、皮肤瘀点 30年 ,于 1992年 7月入院。查体 :贫血貌、颈部及下肢皮肤散在出血点 ,肝、脾肋下未触及。血常规 :红细胞2 9× 10 12 /Ｌ ,血红蛋白 6 6ｇ/Ｌ ,白细胞2 4× 10 9/Ｌ ,中性粒细胞 0 34,淋巴细胞 0 6 4,单核细胞 0 0 2 ,血小板 2 1×10 9/Ｌ ,网织红细胞 0 0 0 2。骨髓 :有核细胞增生减低 ,粒系、红系减少 ,巨核细胞未见。中性粒细胞碱性磷酸酶 (ＡＬＰ)积分 :阳性指数 395 ,阳性率 10 0 %。骨髓活检 :增生极度低下 ,造血组织 0 15 ,脂肪组织 0 85 ,粒系、红系减少 ,巨核细胞未见。Ｃｏｏｍｂｓ…     

骨髓增生异常综合征伴巨核细胞发育异常3例报告

目的:探讨骨髓增生异常综合征(MDS)伴巨核细胞发育异常[又称难治性血小板减少型MDS(MDSRT)]患者的临床特点及与特发性血小板减少性紫癜(ITP)的鉴别要点。方法:回顾性分析3例MDS-RT的临床特点、诊治经过、骨髓凃片小巨核细胞酶标(CD41a)免疫组化染色及骨髓细胞遗传学的结果。结果:3例血小板计数显著减少,骨髓像巨核细胞增生伴成熟障碍,其中,2例可见单圆核巨核细胞,临床上初始诊断为ITP,但用激素治疗、甚至环胞素治疗,无明显疗效。最后2例发现细胞遗传学异常,1例骨髓凃片CD41a免疫化学染色发现巨核细胞呈明显的病态造血,诊断为MDS-RT。结论:对难治性血小板减少性紫癜患者,应做骨髓活检,骨髓巨核细胞CD41a或CD42b免疫化学染色,细胞遗传学检查,注意与MDS-RT的鉴别。     

62例系统性红斑狼疮血液学改变及误诊分析

通过６２例系统性红斑狼疮血象、骨髓象及临床资料分析，（１）贫血是ＳＬＥ最常见的症状，其中肾脏损害的盆身最多见，其次为缺铁和自身免疫性溶贫。（２）ＳＬＥ的骨髓象是增生性骨髓象，粒系增生核左移，巨核细胞数量增生良好，但产血小板功能较差，（３）ＳＬＥ可引起血细胞减少易诊为血液病。     

Clinical analysis of primary Sjögren’s syndrome complicating anemia

This is a cross-sectional study to assess the prevalence and causes of anemia in the primary Sjögren’s syndrome (pSS). One hundred and thirty-two consecutive patients with pSS were enrolled into the study. Standard hematological and immunological tests and examination of bone marrow were performed. Anemia occurred in 45 (34.1%) patients. The causes of anemia included anemia of chronic disease (69%), autoimmune hemolytic anemia (AIHA, 18%), iron deficiency anemia (9%) and other causes (4%), of which AIHA caused the most severe anemia. The prevalence of ANA, anti-Ro/SSA, and anti-La/SSB was much higher in patients with anemia than those without anemia. Anticardiolipin antibodies were most commonly detected in AIHA; the prevalence of IgG and hypocomplementemia in AIHA was much higher in patients without anemia. Abnormal bone marrow changes were observed in two cases with anemia, one with morphological changes in the myeloid, megakaryocytic, and erythroid lineages and one with hypocellularity in the erythroid lineage. Therefore, pSS patients with anemia may be associated with destruction of peripheral mature blood cells, impaired red cells production, and hematopoietic abnormalities due to an immune mechanism, although the concrete pathogenesis is still unclear.     

巨核细胞酶标染色法在贫血鉴别诊断中的应用价值

目的：探讨酶标法检测骨髓小巨核细胞在贫血诊断和鉴别诊断中的意义。方法：单纯性贫血患者共计123例，其中，骨髓增生异常综合征26例，缺铁性贫血20例，巨幼细胞性贫血10例，溶血性贫血18例，多发性骨髓瘤21例，骨髓纤维化9例，肾性贫血7例，纯红细胞再生障碍性贫血3例，慢性病贫血5例，慢性再生障碍性贫血4例。分别应用巨核细胞酶标染色法（APAAP）和瑞特染色法，计数患者骨髓涂片中巨核细胞的总数（包括小巨核细胞），并对小巨核细胞进行分类计数。结果：APAAP法较瑞特染色法检测的巨核细胞的数量多，差异有统计学意义（P〈0．05）；且APAAP法较瑞特染色法检测小巨核细胞的阳性率高，差异有统计学意义（P〈0．05）。结论：APAAP法检测骨髓小巨核细胞，其结果明显优于瑞特染色法，阳性率高且特异性强，在单纯性贫血的鉴别诊断中有重要的应用价值。     

Evaluation of erythrocyte basic ferritin in the diagnosis of anemia

Erythrocyte basic ferritin (EF) concentration was determined in 64 normal subjects, 123 patients with anemia and 12 patients with leukopenia and thrombocytopenia. There was a significant difference between males and females. Other iron indices, including plasma iron (PI), total iron binding capacity (TIBC), zinc protoporphyrin (ZnPP) and plasma ferritin (PF) were also determined in all the subjects and bone marrow iron stain was determined in the 135 patients. The lowest EF concentration was seen in patients with iron deficiency anemia, being significantly lower than that in normal subjects. EF concentration in patients with iron deficiency erythropoiesis was also lower than that in normal subjects and at the same time significantly different from that in patients with iron deficiency anemia. EF concentration increased prior to PF concentration in patients with iron deficiency anemia who had been treated for a period of 1-8 weeks. EF concentration in patients with anemia of chronic diseases had a significant difference as compared with that in normal subjects and in patients with iron deficiency anemia, but EF concentration in those patients who were accompanied by iron deficiency was similar to that in patients with simple iron deficiency anemia. EF concentration in some iron overloaded patients (aplastic anemia, megaloblastic anemia, MDS etc.) was significantly higher than that in normal subjects. It was demonstrated that there was a good correlation between EF concentration and bone marrow sideroblastic iron in the rank correlation analysis of the iron indices in 135 patients (rs 0.893, P less than 0.01). PF concentration had the best correlation with marrow iron (rs 0.948, P less than 0.01).     

骨髓增生异常综合征MIC异常和临床特点

目的：研究骨髓增生异常综合征（MDS）形态学、免疫学、遗传学（MIC）异常变化及临床特点.方法：对65例MDS患者的血液学、免疫学、遗传学异常改变资料进行了回顾性分析.结果：65例中外周血象全血细胞减少36例（55.4%），2系细胞减少19例（29.2%），分类可见幼红细胞35例（53.8%），幼粒细胞27例（41.5%）.贫血60例（92.3%），以中重度为主58例（89.2%），表现为大细胞或正细胞性贫血.白细胞异常56例（86.2%），以减少为主45例（69.2%）.血小板减少41例（63.1%）.65例行骨髓细胞学检查：增生活跃至极度活跃55例（84.6%），1系或1系以上病态造血54例（83.1%），11例难治性质血患者病态造血不明显（16.9%）.59例行骨髓病理学检查：3系不同程度病态造血30例（50.8%），粒系幼稚前体细胞异常定位（ALIP）29例（49.2%）.45例行骨髓细胞流式细胞仪（FCM）免疫表型测定，表现2系或2系以上异常40例（88.9%）.41例做FCM-DNA倍体分析，检出DNA非整倍体26例（63.4%）.32例做骨髓细胞染色体分析，发现异常克隆13例（40.6%）.结论：MDS临床表现不典型，血液学改变复杂，缺乏特异性，部分RA病态造血不明显，仅依靠形态学难以做出正确诊断，应提倡MIC分型诊断。     

艾滋病的临床及血液学特点

目的 :研究艾滋病的临床及血液学特点。方法 :总结在我院确诊并有详细血液学资料的 15例患者 ,分析其临床表现、免疫功能及血液学特点。结果 :15例患者中发热 13例 ( 86 .7% ) ,淋巴结肿大 10例 ( 6 6 .7% ) ,消瘦 6例 ( 4 0 .0 % ) ,真菌感染 6例 ( 4 0 .0 % ) ,白细胞减少 8例 ( 5 3.3% ) ,贫血 12例 ( 80 .0 % ) ,为正常细胞正常色素性贫血 ,血小板减少 6例 ( 4 0 .0 % ) ,骨髓有核细胞增生、浆细胞增多及病态造血 ,免疫学检查主要为细胞免疫缺陷。结论 :艾滋病患者存在免疫功能低下 ,其临床、血液学和骨髓改变有一定特点     

Excess alcohol consumption is common in patients with cytopenia: studies in blood and bone marrow cells

BACKGROUND: Although alcohol abuse is known to create a variety of adverse effects on hematopoiesis, the associations between ethanol consumption and hematological abnormalities have not been fully established. METHODS: We studied 144 consecutive adult patients who underwent clinical and bone marrow examinations due to abnormal findings in peripheral blood cell counts or red blood cell indices without previously established diagnoses of specific hematological diseases, malignancies, or infections. Assessment included the amount of alcohol consumption, complete blood cell counts, morphological review of peripheral blood and bone marrow, markers of liver status, and erythrocyte folate and serum vitamin B12 levels. RESULTS: There were 57 (40%) patients who showed a history of hazardous drinking and 87 patients who were either nondrinkers or social drinkers. The incidence of anemia was 51% in the alcohol abusers, as compared with 69% of the nonalcoholics (p < 0.05). A diverse pattern of hematological effects was observed in the alcohol abusers. Abnormal platelet and leukocyte levels were common, especially in the anemic alcoholics. Both increased mean cell volume of erythrocytes (macrocytosis; 67 vs. 18%; p < 0.0001) and mean cell hemoglobin (63 vs. 22%; p < 0.0001) were more frequent in the alcoholics than in the nonalcoholics. Reticulocytosis (37%), thrombocytopenia (41%), and combined cytopenias (34-38%) were also common findings in the alcoholic patients. The blood smears from such patients typically showed round macrocytes, stomatocytes, and knizocytes. Bone marrow aspirates revealed vacuolization of pronormoblasts in 24% of the alcoholic patients. Interestingly, megakaryocytes in the cell periphery were also vacuolized in 20% of the alcohol abusers, especially in those with recent intoxication. CONCLUSIONS: Our findings suggest that alcohol abuse results in diverse patterns of hematological effects and affects several cell lines. Therefore, in patients undergoing bone marrow examinations due to cytopenias, the probabilities for likely findings seem to be different between alcoholics and nonalcoholics. Information on ethanol consumption should be systematically included in the clinical assessment of such patients.     

Bone marrow status of anaemic pregnant women on supplemental iron and folic acid in a Nigerian community

The bone marrow status of 31 consecutive pregnant women who had been on supplemental oral iron and folic acid since early pregnancy at the University of Benin Teaching Hospital was assessed later in pregnancy to test the efficacy of oral iron and folic acid in preventing iron deficiency and/or megaloblastic anaemia in our community. Only those pregnant patients with haemoglobin genotype AA or AS took part in the study. Nobody was excluded except those with CC or SC. 96.77% (30 out of 31 patients) had iron deficiency with no stainable iron in the bone marrow. 35.4% (11 out of 31 patients) had megaloblastic changes in the bone marrow. 32.2% (10 out of 31 patients) had a combined iron deficiency and megaloblastic anaemia while only one out of 31 patients (3.23%) had megaloblastic anaemia without concurrent iron deficiency. 60.4% (20 out of 31 patients) had iron deficiency alone without concomitant megaloblastic changes in marrow. The bone marrow in all the patients were normal in other respects except with regards to iron-deficiency and/or megaloblastic status. The significance of this high incidence of iron-deficiency and/or megaloblastic anaemia in patients already on routine pre-natal drugs is discussed.     

Significance of Haematological Parameters in the Non-Hodgkin''s Malignant Lymphomas

Blood findings at diagnosis, in 140 adults with lymphoma, were correlated with bone marrow involvement and survival. An abnormal haemoglobin, leucocyte count or platelet count was found in 57% of patients. Lymphocytopenia occurred in 46%. All patients with thrombocytopenia or neutropenia, 69% with leucopenia and 63% with anaemia had marrow involvement with lymphoma. Marrow involvement in histiocytic and stem cell lymphoma was always associated with anaemia. Marrow involvement in poorly differentiated lymphocytic lymphoma (PDL) was associated with anaemia, thrombocytopenia, leucopenia, lymphocytopenia or lymphoma cells in the blood in 93% of patients. Bone marrow involvement was found in only 13% of patients with normal haematological parameters. In the absence of marrow involvement blood abnormalities at diagnosis did not generally correlate with survival. However, among patients with diffuse PDL who had marrow involvement, anaemia, thrombocytopenia and leucopenia adversely affected survival. Lymphocytopenia did not correlate with survival.     

Post-gastrectomy anemia: evaluation of 72 cases with post-gastrectomy anemia

Anemia is common in patients following gastrectomy. The purpose of this study was to document causes of anemias developing during the post-gastrectomy period and to determine the importance of complete blood count parameters on types of anemia. A total of 72 patients (23 women and 49 men) who had previously undergone gastrectomy in the past and who were admitted for the evaluation of anemia were enrolled in study. The patients who were evaluated and treated for anemia in the post-gastrectomy period were excluded. Iron deficiency anemia was present in 68 (94.4%) of 72 gastrectomized patients with anemia. Deficiencies of vitamin B12 and folate were present in 57 (79.2%) and in three patients, respectively. The most common cause of anemia was the combination of iron and vitamin B12 deficiencies. Iron deficiency was present in the majority of patients, followed by vitamin B12 deficiency in frequency. In all combinations of iron deficiency, the values of mean cell hemoglobin and mean cell hemoglobin concentration were either normal or low. In cases who had low white blood cell and platelet counts vitamin B12 deficiency was frequent, while in cases who had high numbers of white cells or platelets iron deficiency was more frequent. In conclusion, gastrectomized patients should be followed for anemia and treated appropriately based on the cause of anemia.     

Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias

We studied 21 bone marrow specimens from 21 patients with systemic lupus erythematosus (SLE) and peripheral cytopenias: anaemia (Hb<10 g/dl), and/or leucopenia (white blood cell count <4×10⁹/l), and/or thrombocytopenia (platelets <150×10⁹/l). None of the patients had used immunosuppressive drugs in the 2 months before the study, and 11 (52.4%) had never used these drugs. The global and specific series cellularity, degree of fibrosis and necrosis were evaluated by bone marrow trephine; morphological abnormalities and iron stores were evaluated by cytological smears. The most important abnormalities viewed in bone marrow biopsies were: global hypocellularity (47.6%), increased reticulin proliferation (76.2%) with myelofibrosis in one patient, and necrosis (19.0%). The marrow aspirates were difficult to obtain in four patients, who showed an increased reticulin proliferation on histological analysis. Plasmocytosis was present in 26.7% of cases and in one there was a serum monoclonal component (IgG kappa). Iron stores were normal or increased in 26.7% of specimens and decreased or absent in 73.3%. The most frequent peripheral abnormality was leucopenia in 90.4% (19/21) and granulocytic hypoplasia was observed in 47.3% (9/19) of these patients. We conclude that the bone marrow may be a target organ in SLE with cytopenias.