Congenital sideroblastic anemia treated as thalassemia major

Sideroblastic anemia is a rare cause of microcytic hypochromic anemia. In Bangladesh, most common causes of microcytic anemia are iron deficiency anemia, anemia of chronic diseases and thalassemia. Serum ferritin is usually done to differentiate them. If serum ferritin is low, the diagnosis of iron deficiency is entertained. When serum ferritin is raised but erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are normal - anemia of chronic disease is excluded. The next investigation is Hb-electrophoresis. Normal Hb-electroporesis excludes thalassemia. Then bone marrow examination with iron stain is done for the diagnosis of sideroblastic anemia. Here we report a case of a 14 year old girl presenting with intermittent leg pain and anemia. Her blood flim showed microcytic hypochromic anemia with raised serum ferritin and normal Hb-electroporesis. Initially she was labeled as a case of unusual type of thalassemia and treated with blood transfusion. Finally bone marrow examination with iron stain was done and she was diagnosed as a case of congenital sideroblastic anemia. We reviewed the literature and discussed the management as well.     

30例血液学异常的2型糖尿病患者骨髓象分析

目的观察血液学异常的糖尿病患者的骨髓象特征,探讨其临床价值和意义。方法选取血液学异常的30例2型糖尿病患者进行骨髓穿刺,并对骨髓象进行分析。结果 30例糖尿病患者中骨髓诊断为粒系核左移伴继发性贫血17例(56.7%),缺铁性贫血3例(10.0%),铁粒幼红细胞性贫血1例(3.3%),溶血性贫血1例(3.3%),血小板减少性紫癜2例(6.6%),白血病4例(3.3%),多发性骨髓瘤1例(3.3%),干抽1例(3.3%)。结论通过骨髓检查有助于了解2型糖尿病合并血液学异常的病因和发病机制,帮助临床医生及时准确地对症治疗。     

PATHOLOGICAL CHANGES IN RED CELL SERIES ELECTRON MICROSCOPIC STUDY ON BONE MARROW CELLS OF SOME BLOOD DISORDERS

In 26 cases of aplastic anemia, iron deficiency anemia, hemoglobinopathy, paroxysmal nocturnal he. inoglobinemia, sideroblastic anemia and erythroleu- kemia, the ultrastructural changes in the red cell series were studied. The disease had some similar structural changes such as ropalocytosis of cell membrane, iron loaded mitochondria and clear-cut demarcation between the heterochromatin and euchromatin, although their causes or pathogenesis are different. Numerous red ceUs with inclusion bodies were found in one case of PNH after splenec tomy.     

贫血患者血清铁铁蛋白细胞内外铁检测结果分析

目的探讨各类贫血患者血清铁、铁蛋白和骨髓细胞内外铁含量多或寡与贫血症的相互关系.方法应用比色法、放射免疫法和普鲁士兰反应法检测186例各种贫血患者血清铁、铁蛋白浓度和细胞内外铁粒、铁小珠的含量.结果急、慢性白血病贫血、再生障碍性贫血、铁粒幼红细胞性贫血、溶血性贫血、巨幼红细胞性贫血患者血清铁浓度升高;肾性贫血、肝性贫血、缺铁性贫血、肿瘤性贫血、骨髓增生异常综合症(MDS-RA)患者血清铁下降;铁蛋白增高主要见于急、慢性白血病贫血和铁粒幼红细胞性贫血、肿瘤性贫血及骨髓增生异常综合症等;细胞内外铁颗粒减少和外铁消失主要见于缺铁性贫血和肾性贫血.结论检测血清铁、铁蛋白和细胞内外铁能灵敏地反映机体铁的贮存和利用水平;恶性肿瘤或其它细胞快速增殖时,细胞合成铁蛋白的能力较强.     

继发性铁粒幼细胞性贫血铁代谢诊断指标研究应用

[目的 ]探讨研究继发性铁粒幼细胞性贫血铁代谢状况及发病机理 ,开发铁粒幼贫血早期诊断及预防方法。 [方法 ]利用骨髓五铁 (即细胞内铁、细胞外铁、病理环铁粒幼细胞、铁粒幼细胞比率 (SR)、铁粒红细胞 ) ;血清四铁 (血清铁、总铁结合力、未饱和铁、饱和度 )上述各项指标 ,对 1 4 2 82例各种不同血液病患者进行检测。 [结果 ]原发性铁粒幼细胞贫血 30例 ;继发性铁粒幼细胞贫血93例 ,可继发于 2 0多种疾病 ,化疗后白血病 2 8例 (30 .1 % ) ;除缺铁贫外各种贫血 4 3例 (4 6 .2 % ) ;其它感染、粒细胞减少、骨髓纤维化、真性红细胞增多症 2 2例 (2 3.7% ) ;原发铁粒幼细胞贫血病理环铁粒幼细胞体积小 ,铁颗粒数目多 ,常如黄豆粒似的撒在核周围 ,病理环铁粒幼细胞数目增多 >30 % ,此点与骨髓增生异常综合症 (MDS -RAS)不同 ,该病病理环铁粒幼细胞颗粒数目少 ,并出现病态造血巨幼细胞浆带中 ,铁粒幼细胞≥ 1 5 %。[结论 ]骨髓五铁、血清四铁各项指标 ,对原发性、继发性铁粒幼细胞贫血以及MDS、RA -S的诊断、鉴别诊断、预防有重要意义 ,对输血补铁有重要价值。     

血清铁蛋白测定的临床应用

本文报道血清铁蛋白测定33种儿科疾病,计103例。以观察临床各种疾病的血清铁蛋白值并应用于临床诊断。通过检测结果,提示急性白血病、铁粒幼细胞性贫血和再生障碍性贫血的血清铁蛋白值明显增高以及肺炎、败血症等感染性疾病的血清铁蛋白随亦高于正常。营养性缺铁性贫血的血清铁蛋白值则明显减少。     

儿童小细胞低色素性贫血与全血中铜、锌、钙、镁、铁、铅元素关系的探讨

目的探讨小儿小细胞低色素性贫血与全血铜、锌、钙、镁、铁、铅金属元素的关系。方法对2011年4月～2012年4月收治的60例小细胞低色素性贫血患儿做为病例组,60例健康小儿做为正常对照组(年龄、身高、体重、营养状况无统计学差异),分别采血,利用原子吸收分光光度法检测两组儿童全血中铜、锌、钙、镁、铁、铅六种金属元素的含量。结果病例组儿童全血中铁、锌、镁含量低于正常组,差异有统计学意义(P<0.05);铜、钙病例组和健康对照组差异无统计学意义(P﹥0.05);铅含量病例组高于正常对照组且差异有统计学意义(P<0.05)。结论小儿小细胞低色素性贫血与全血铁、锌、镁元素缺乏和全血铅元素增高有关。     

160例贫血患者的血液检验结果分析

目的对贫血患者的血液检验结果进行分析。方法选取1 60例贫血患者,同时,再选择50例健康体检者为对照组,对160例贫血患者和健康体检者用一次性真空采血管来进行静脉采血5 mL。记录所有血液标本中红细胞分布宽度（red cell distribution width,RDW）、平均红细胞血红蛋白浓度（mean corpuscular hemoglobin concentration,MCHC）、平均红细胞血红蛋白量（mean corpuscular hemoglobin,MCH）、平均红细胞容积（mean corpuscular volume,MCV）的检测结果。结果慢性感染、地中海贫血、缺铁性贫血、巨幼细胞性贫血、溶血性贫血患者的RDW、MCHC、MCH、MCV与健康对照者相比,差异有统计学意义（P〈0.05）。结论贫血并非一种具体疾病,而是由各系统疾病引起,多种不同原因的贫血症都会导致出现类似的临床表现和血液学特征,因此,需要通过RDW、MCHC、MCH、MCV这些常规检验指标来鉴别诊断贫血患者。     

12例肾性贫血误诊原因的分析

有些慢性肾脏疾病引起的贫血,由于患者病史或临床上缺乏典型表现,一般尿常规的发现又不显而未加注意,或同时伴发可以解释贫血的种种疾病,遂使慢性肾病性贫血长期未能得到正确诊断,对这类患者,包括经铁剂治疗一度见效的病人,尿常规的轻微变化不可忽视,必要的肾功能检查不可放弃     

幼儿营养调查及发锌发钙的相关分析

对市内两所幼儿园215名幼儿进行了营养调查及发锌、发钙的测定。调查结果表明,受检幼儿的一般体格发育状况良好,但贫血和佝偻病症罹患率较高,分别为22.5%和52.0%。幼儿膳食摄入的蛋白质、热能、维生素B_1、B_2、PP及A的平均水平接近或达到了标准供给量,铁的供给量虽高,但优质来源少;钙、锌的摄入则严重不足。幼儿发锌发钙的相关分析表明,发锌与发钙值呈正相关,有佝偻病症者发锌值低于无佝偻病症者,提示维生素D可能参与体内锌代谢的调节。     

再生障碍性贫血血清及红细胞内锌含量的研究

用原子吸收分光光度法测定了48例再生障碍性贫血(再障)患者血清及红细胞内锌的含量.血清锌含量明显低于正常人(P<0.01),红细胞内锌含量高于正常人(P<0.05),血清锌与红细胞锌呈负相关(r为-0.532,P<0.01).病情缓解时,红细胞内锌含量接近正常,红细胞内锌含量变化出现于骨髓变化的同时或之后,血象尚无明显变化之前.结果提示:再障时,血液成分中锌分布异常,红细胞锌水平对估计再障病情变化可能有一定帮助.     

Zinc metabolism in sickle cell anemia.

Zinc metabolism was studied in 34 patients with sickle cell anemia in a steady state and in six patients during sickle cell crisis. The patients in steady state were found to have significantly lower plasma levels and higher urinary levels of zinc than the 50 healthy adults in the control group. During sickle cell crisis the plasma zinc levels dropped to a mean value of 0.54 microgram/microL. These results support the concept that zinc deficiency occurs in sickle cell anemia and that further drops occur in the serum zinc levels during sickle cell crisis.     

Marrow failure with sideroblasts in an autoimmune haemolytic crisis

We report a case of autoimmune haemolytic anaemia complicated by ineffective erythropoiesis associated with florid sideroblastic change in the bone marrow.     

Sideroblastic and megaloblastic change

A case of primary sideroblastic anaemia is described in which megaloblastic changes in the bone marrow were found to be due to coincidental pernicious anaemia.     

Element of caution: a case of reversible cytopenias associated with excessive zinc supplementation

ZINC IS A COMMON SUPPLEMENT AND IS WIDELY AVAILABLE as a standard component of many over-the-counter products. A number of reports have identified an association between excessive zinc intake and severe cytopenia. We report a case of zinc-induced copper deficiency in a young adult to illustrate this under-recognized cause of anemia and neutropenia.     

Hypocupremia induced by zinc therapy in adults.

Hypocupremia occurred in an adult with sickle cell anemia who received zinc as an antisickling agent for two years. The hypocupremia was associated with microcytosis and relative neutropenia. Administration of copper resulted in an increase in RBC size and leukocyte counts. We have since observed hypoceruloplasminemia of varying degrees in several other sickle cell anemia patients who were receiving oral zinc therapy. This complication was easily corrected by copper supplementation.     

以“芪英三两三”组方经验为指导治愈顽固慢性再生障碍性贫血1例

慢性再生障碍性贫血属于临床血液系统常见疾病之一，发病率呈逐年升高趋势，病程长，易反复，对患者的生活质量形成严重影响。姜苗教授认为慢性再生障碍性贫血不仅与肾虚相关，且与脾胃升降功能联系密切，强调从脾论治，抓住“虚”“瘀”“毒”3个病理因素，活用“芪英三两三”组方加减，健脾益肾，化瘀解毒。文章以完全治愈1则顽固慢性再障合并继发血小板减少病案为例，探讨姜教授治疗慢性再生障碍性贫血思路及用药方案。     

Dactylitis in a child with sickle cell trait

Dactylitis commonly occurs in patients with homozygous hemoglobin S disease (sickle cell anemia), sickle cell-hemoglobin C disease or sickle cell-beta-thalassemia. A case is reported of dactylitis associated with sickle cell trait, a very rare occurrence. It may be that in this patient the disorder was secondary to severe diarrhea and dehydration.     

骨髓增生异常综合征血清铁蛋白检测的临床意义

目的　探讨骨髓增生异常综合征 (myelodysplasticsyndromes ,MDS)血清铁蛋白检测与预后的关系。 方法　采用液相平衡竞争放射免疫分析法 (radio immunityanalysis,RIA)对 6 1例MDS患者进行血清铁蛋白检测 ,并进行追踪随访观察。结果　环形铁粒幼红细胞增多性难治性贫血 (refractoryanemiawithringsideroblasts,RAS)、原始细胞增多性难治性贫血 (refractoryanemiawithexcessofblasts,RAEB)、转化型原始细胞增多性难治性贫血 (refractoryanemiawithexcessofblastsintransformation ,RAEB T)、慢性粒 单核细胞白血病 (chronicmyelomonocyticleukemia ,CMML)中血清铁蛋白 (serumferritin ,SF)均明显增高 ,与对照组比较差异有显著性。难治性贫血 (refractoryanemia ,RA)中有 7例的血清铁蛋白升高 ,最高值为 2 30 μg/L ,但无一例转化为急性白血病。但是RAEB、RAEB T的SF均大于 4 0 0 μg/L ,差异有显著性 ,尤其是RAEB T的SF最高值达 92 0 μg/L ,且都转化为急性白血病 ,死亡率高达 83.3%。死亡组和转化为AL组的SF均明显升高 ,差异有显著性。结论　血清铁蛋白水平与MDS转化为急性白血病呈正相关 ,且与死亡率有关。