Thrombotic thrombocytopenic purpura without schistocytes on the peripheral blood smear

A hallmark of the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) is evidence of microangiopathic hemolytic anemia. The presence of schistocytes on the peripheral blood smear, elevated plasma lactic dehydrogenase, and decreased haptoglobin concentration are used as evidence of microangiopathic hemolytic anemia to make a diagnosis of TTP. This report describes a case of recurrence of TTP in the absence of schistocytes in the peripheral blood smear during the recurrent episode. Although careful attention should be paid to microscopic examination of a blood smear in any patient presenting with acute renal failure and thrombocytopenia, this case emphasizes the need to consider TTP-hemolytic uremic syndrome in the differential diagnosis, even in the absence of peripheral schistocytosis.     

Thrombotic thrombocytopenic purpura

This serious disorder of young adults is characterized by fever, hemolytic anemia, hemorrhagic signs, various neurologic abnormalities and renal dysfunction. Laboratory findings include a negative Coombs' test for hemolytic anemia, severe thrombocytopenia, and fragmented erythrocytes on the peripheral blood smear. Without treatment, mortality may exceed 80 percent. Early diagnosis and prompt therapy with high-dose corticosteroids and antiplatelet agents, as well as exchange plasmapheresis when indicated, bring about remission in 60 to 80 percent of patients.     

Abdominal pain and sicklemia in a patient with sickle cell trait

We have presented a case of thrombotic thrombocytopenic purpura initially misdiagnosed as sickle cell crisis. Based on the "history" of hemoglobin S disease, anemia, and the presence of apparent sickled cells on the peripheral blood smear, the diagnosis of sickle cell crisis seemed reasonable. The patient is described because of unusual features of the case, to emphasize again a hazard of sickle cell screening and the value of reviewing the peripheral blood smear in cases of anemia.     

Extramedullary blast crisis and hemolytic anemia in a patient with t(3p+;4q-) and rapidly evolving myelogenous leukemia

We have reported the case of a patient with a rapidly evolving myelogenous leukemia associated with hemolytic anemia, extramedullary evolution, and an isolated translocation of the long arm of chromosome 4 to the short arm of chromosome 3. The hemolytic process was primarily extravascular and was not associated with pyruvate kinase or glucose-6-phosphate dehydrogenase deficiency. Although blasts were absent from the peripheral smear and represented less than 10% of the bone marrow myeloid precursors, multiple organs including the heart, liver, lungs, and spleen were infiltrated with blasts at autopsy.     

Hemolytic anemia

Hemolysis presents as acute or chronic anemia, reticulocytosis, or jaundice. The diagnosis is established by reticulocytosis, increased unconjugated bilirubin and lactate dehydrogenase, decreased haptoglobin, and peripheral blood smear findings. Premature destruction of erythrocytes occurs intravascularly or extravascularly. The etiologies of hemolysis often are categorized as acquired or hereditary. Common acquired causes of hemolytic anemia are autoimmunity, microangiopathy, and infection. Immune-mediated hemolysis, caused by antierythrocyte antibodies, can be secondary to malignancies, autoimmune disorders, drugs, and transfusion reactions. Microangiopathic hemolytic anemia occurs when the red cell membrane is damaged in circulation, leading to intravascular hemolysis and the appearance of schistocytes. Infectious agents such as malaria and babesiosis invade red blood cells. Disorders of red blood cell enzymes, membranes, and hemoglobin cause hereditary hemolytic anemias. Glucose-6-phosphate dehydrogenase deficiency leads to hemolysis in the presence of oxidative stress. Hereditary spherocytosis is characterized by spherocytes, a family history, and a negative direct antiglobulin test. Sickle cell anemia and thalassemia are hemoglobinopathies characterized by chronic hemolysis.     

Cerebral falciparum malaria mimicking thrombotic thrombocytopenic purpura

We have described a patient with cerebral falciparum malaria who had rapidly progressive CNS deterioration, renal failure, hemolytic anemia associated with striking and varied erythrocyte morphologic changes, and thrombocytopenia. The initial diagnosis was thrombotic thrombocytopenic purpura (TTP) of unknown origin. Reexamination of the peripheral smear of this comatose patient led to correct diagnosis and effective treatment in this case of cerebral falciparum malaria--another of medicine's great mimickers.     

Diagnostic d’une anémie hémolytique en réanimation

Hemolytic anemia is not an exceptional situation in adults. The abrupt onset of hemolysis and the severity of anemia may sometimes be life-threatening and require admission to the intensive care unit. Establishing the hemolytic mechanism of an anemia is rather easy, but finding its etiology can be quite difficult. The diagnosis of severe hemolytic anemia requires a rapid multiple-step procedure taking into account patient and family history, a careful analysis of the blood smear as well as the result of a direct antiglobulin test. While management is mainly supportive, some causes of hemolytic anemia may require “specific” and emergent therapy, based only on a rapid diagnosis procedure.     

Acute colitis resembling ulcerative colitis in the hemolytic-uremic syndrome.

The case report of a 10-year-old boy, admitted to the hospital after he had experienced 4 days of periumbilical abdominal pain, intermittent vomiting, and diarrhea, is presented. He had proctoscopic and radiologic findings resembling ulcerative colitis. However, further analysis of laboratory data suggested hemolytic-uremic syndrome. Since the patient in the pediatric age group presents with a clinical picture mimicking ulcerative colitis, this hemolytic-uremic syndrome should be included in the differential diagnosis. Examination of a peripheral smear revealing typical findings of microangiogpathic, hemolytic anemia, thrombocytopenia, and a rising blood urea nitrogen value will lead to the diagnosis of hemolytic-uremic syndrome and early appropriate therapy.     

系统性红斑狼疮相关性血液病血液学异常临床特点

为了研究系统性红斑狼疮相关性血液病(SLERHD)患者血液学异常的临床特点,探讨鉴别诊断的依据,采用SPSS/PC软件对92例SLERHD患者的血液学检查资料进行回顾性分析总结。结果发现:此类患者临床表现及血像缺乏特异性,但所有病人都具备SLE相关的多种自身抗体阳性、血清球蛋白增高及不同程度的皮肤、关节症状。以贫血为首发症状者67例(72.8％),以皮肤粘膜紫癜为主要表现者39例(42.4％),以溶血性贫血为首发症状者17例(18.5％),白细胞减少56例(60.9％),血小板减少54例(58.7％),全血细胞减少者41例(44.6％)。骨髓有核细胞增生良好,其中活跃者57例(61.9％),明显活跃者35例(38.1％);粒/红(G/E)多数正常,>3者59例(64.1％),<3者33例(35.9％),17例溶血性贫血者Coombs试验均为阳性,G/E<1;巨核细胞增多者80例(86.9％),正常者11例(11.9％),<7个/片者1例(1.1％),中性粒细胞硷性磷酸酶(NAP)积分均为阴性。结论:虽然SLERHD临床表现多种多样、血像缺乏特异性,但所有病人都具备SLE相关的多种自身抗体阳性、血清球蛋白增高及不同程度的皮肤关节症状,属于增生性贫血,Ret,G/E和骨髓粒、红、巨核三系细胞数增多或正常,NAP阴性,可与再生障碍性贫血(AA)鉴别;细胞形态基本正常、无病态造血可与骨髓增生异常综合征(MDS)鉴别;多种自身抗     

An unusual case of autoimmune hemolytic anemia with reticulocytopenia, erythroid dysplasia, and an IgG2 autoanti-U

BACKGROUND: Autoantibodies with anti-U specificity, usually in combination with autoantibodies of other specificities, have occasionally been identified in association with autoimmune hemolytic anemia. A case of life-threatening autoimmune hemolytic anemia, characterized by several atypical features, including apparent intravascular hemolysis associated with an IgG2 anti-U, reticulocytopenia, and bone marrow dyserythropoiesis is described. CASE REPORT: A 36-year-old man with a severe case of acute-onset autoimmune hemolytic anemia was admitted to another hospital; he had a hematocrit of 15 percent, elevated bilirubin and lactate dehydrogenase, and positive direct and indirect antiglobulin tests. He received 7 units of incompatible red cells without improvement in hematocrit, and he was transferred to University Hospitals of Cleveland (OH). He was jaundiced and became syncopal in the sitting position. His serum was reddish pink; he had a hematocrit of 11.8 percent and a reticulocyte count of 2.5 percent. No spherocytes were observed in the peripheral blood smear. Shortly after admission, the hematocrit fell to 6.9 percent. He was given 3 units of “least-incompatible” red cells and was started on prednisone, with little improvement. An IgG2 autoanti-U was detected in his serum. Seven units of U- red cells were transfused over the next 4 days. The hematocrit improved to 23 percent and continued to rise without further transfusion. A bone marrow examination, initially revealing erythroid hyperplasia accompanied by dyserythropoiesis, became morphologically normal. Drug studies failed to show evidence of drug-related hemolysis. He remains well 2 years after discharge without evidence of recurrent hemolysis. CONCLUSION: Severe life-threatening autoimmune hemolytic anemia, in this instance induced by an autoanti-U, may be associated with IgG2 autoantibody and characterized by apparent intravascular hemolysis and bone marrow dyserythropoiesis. Early treatment with U- blood, in addition to steroids, may be beneficial.     

Extra-intestinal complications of ulcerative colitis: hematologic complication

Iron deficiency anemia, autoimmune hemolytic anemia, folic acid deficiency megaloblastic anemia, granulocytopenia, acute or chronic leukemia, idiopathic thrombocytopenic purpura have been reported as blood diseases among the extra-intestinal complications with ulcerative colitis until now. Iron deficiency anemia is most frequently seen, and it often derives from apparent or inapparent continuous gastrointestinal bleeding. Autoimmune hemolytic anemia produces antierythrocyte membrane autoantibody while idiopathic thrombocytopenic purpura produces antithrombocyte autoantibody leading to anemia or thrombocytopenia. For folic acid deficiency megaloblastic anemia and granulocytopenia, adverse reaction of sulfasalazine being administered to the patients with ulcerative colitis has been pointed out. While the cases with acute or chronic leukemia are reported increasingly, its cause is still unknown. For treatment of ulcerative colitis, it is considered necessary to find blood complications by carrying out general examinations of peripheral blood and examinations of blood picture, serum iron and folic acid routinely.     

骨髓转移癌22例临床及血液学特点分析

本研究观察和分析了22例骨髓转移癌的临床及血液学特点,并对其进行骨髓细胞形态学、骨髓活检及其他辅助检查。骨髓转移癌首发或主要临床表现为：贫血17例（77.3%)。骨痛10例（45.5%),发热8例（36.4%)。出血4例（18.2%),合并溶血性贫血4例（18.2%)。经辅助检查及病理学检查确诊原发肿瘤为;胃癌6例（27%）。肺癌3例（13.6%)。卵巢癌2例（9%）,乳腺癌、前列腺癌,骨肿瘤,转移性恶性黑色素瘤各1例,原发灶未明7例（31.8%)。血液学变化特点;血红蛋白减少17例（77.3%),血小板减少16例（72.7%),白细胞增多11例（50%）,外周血涂片出现幼粒细胞14例（63.6%),出现幼红细胞9例（40.9%)。Ret增高4例（18.2%)。骨髓涂片在两侧及片尾易见转移癌细胞团,8例骨髓活检证实骨髓腔内肿瘤细胞浸润,细胞呈巢团状分布,磁共振成像（MRI）检查6例有骨质破坏,椎体破坏及异常信号灶。骨髓活检更有助于提高诊断率及确定原发肿瘤的来源,MRI在骨髓转移癌的诊断性检查中具有重要作用。     

自身免疫性溶血性贫血的免疫分型及临床分析

目的 对64例自身免疫性溶血性贫血(AIHA)患者血液中红细胞上结合的抗体进行免疫分型,进一步指导临床治疗.方法 采用免疫分型技术及相关溶血性贫血系列检测法.结果 发病较多的是青壮年,女性多于男性,类型分布以IgG C3型多见,且贫血、溶血程度重,余依次为C3型、IgG型.结论 AIHA免疫分型可判定疾病的严重程度以及为临床治疗提供依据.     

难治性贫血与其它贫血性疾病骨髓及血细胞形态学比较分析

本研究观察骨髓增生异常综合征(MDS),主要是难治性贫血(RA)骨髓及外周血细胞形态学特点,并与其它贫血性疾病(慢性再生障碍性贫血、溶血性贫血、巨幼细胞性贫血)进行比较分析。取患者骨髓及外周血制成涂片行瑞氏染色,骨髓分类500个有核细胞,外周血分类100个有核细胞,观察红系、粒系、巨核系病态细胞特点。结果发现,外周血中性分叶核细胞浆内颗粒稀少或缺如、Pelger核异常改变、幼粒细胞数及检出率、单核细胞检出率以及骨髓粒系各阶段细胞出现颗粒缺如,红系奇数核、核出芽,巨核系小巨核及单圆核巨核细胞等在RA与慢性再生障碍性贫血、溶血性贫血、巨幼细胞性贫血比较均有显著性差异(P<0.05)。结论:细胞形态学是MDS诊断的基础,外周血及骨髓细胞形态学异常在MDS与其他贫血性疾病的鉴别诊断中具有重要作用。     

血细胞减少患者衰变加速因子和膜反应性溶血抑制物表达缺失检测的临床意义

目的检测血细胞减少患者外周血红细胞和中性粒细胞细胞膜糖基磷脂酰肌醇(GPI)连接的补体调节蛋白衰变加速因子(CD55)和膜反应性溶血抑制物(CD59)表达情况,并探讨其临床意义。方法 2006年7月-2011年3月,采用直接免疫荧光标记法流式细胞仪检测182例血细胞减少患者外周血CD55及CD59表达情况,其中阵发性睡眠性血红蛋白尿(PNH)9例,再生障碍性贫血(AA)-PNH综合征8例,AA 83例,骨髓增生异常综合征51例,自身免疫性溶血性贫血11例,造血功能停滞6例,缺铁性贫血7例,巨幼细胞性贫血4例,脾功能亢进3例。结果 PNH及AA-PNH患者CD55、CD59抗原缺失率均较其他血细胞减少者明显增高。结论流式细胞仪检测外周血中红细胞和中性粒细胞膜CD55和CD59抗原表达缺失率是目前诊断PNH可靠和敏感的方法,也是对PNH、AA-PNH早期诊断敏感指标,并且PNH克隆检测还能为诊断疾病提供鉴别诊断依据。     

贫血患者外周血CD55~-和CD59~-细胞检测结果及其意义

采用流式细胞仪检测外周血红细胞和中性粒细胞表达的Glycophosphatidylinositol(GPI)连接的补体调节蛋白CD5 5和CD5 9是目前大多数实验室用来作为诊断阵发性睡眠性血红蛋白尿症 (PNH)的首选实验[1] 。外周血中只要CD5 5、CD5 9阴性细胞占 3%以上流式细胞术即可检出 ,其灵敏度和特异性优于传统的酸溶血实验[2 ] 。我们的研究也证实 ,PNH病人的外周血中均有比例很高的CD5 5 - 和CD5 9- 红细胞和中性粒细胞出现。同时我们也发现在再生障碍性贫血和小细胞低色素性贫血的病人外周血中也有程度不同的CD5 5 - 和CD5 9- 细胞出现 ,现将结…     

儿童不典型再生障碍性贫血与免疫性血小板减少性紫癜的特点及早期鉴别诊断

目的分析并比较不典型再生障碍性贫血(aplastic anemia,AA)和特发性血小板减少性紫癜(idiopathic chrombocytopenic purpura,ITP)的外周血及骨髓特点,为临床诊断提供一定依据,减少误诊的发生。方法选取2008年1月～2014年12月西安交通大学医学院第二附属医院收治的不典型再生障碍性贫血15例为不典型AA组;同期初诊的急性ITP 30例为ITP组。入院后均行血常规、骨髓涂片,自身抗体系列,抗血小板抗体等检查。所有病例入组前排除白血病,MDS,典型AA等血液疾病。结果不典型AA组患儿中性粒细胞计数为2.17±1.07(ANC),血红蛋白为93.23±32.11 g/L(HB)显著低于ITP患儿(ANC为2.73±0.39 g/L及HB为111.11±42.23 g/L),两组间进行t检验,差异具有统计学意义(P<0.05);而ITP患儿血小板计数为(30.38±2.22)×109/L,显著低于不典型再障患儿(47.14±2.17)×109/L,两组间进行t检验,差异具有统计学意义(P<0.05);不典型AA患儿巨核细胞数为17.83±7.83明显低于ITP患儿225.3±64.23,两组间进行t检验,差异具有统计学意义(P<0.05)。结论不典型AA和ITP存在许多相似性,容易发生误诊,当患儿出现血小板降低时,应仔细分析其他血细胞的特点,并参照骨髓穿刺结果,必要时进行骨髓活检以明确诊断。     

Agranulocytosis and hemolytic anemia in patients with renal cell cancer treated with interleukin-12

Interleukin-12 (IL-12) is a cytokine with effects on immune function and hematopoiesis. In this article, the authors describe two patients with renal cell cancer in whom grade 4 neutropenia and grade 3 hemolytic anemia developed, respectively, during treatment with twice-weekly intravenous recombinant human interleukin-12 (rhIL-12) during a phase 1 trial. The severe neutropenia was associated with bone marrow agranulocytosis and a preponderance of large granular lymphocytes in the peripheral blood, whereas the hemolytic anemia was negative for the Coombs test and associated with splenomegaly. The agranulocytosis and hemolytic anemia persisted after the rhIL-12 was stopped, but both subsequently responded to treatment with cyclophosphamide. steroids, or both. These findings indicate that rhIL-12 can induce unique hematologic toxic effects that can be reversed with immunosuppressive drugs.     

Suppressive effect of human interferons on erythroid colony growth in disorders of erythropoiesis

The suppressive effect of two types of human interferon (fibroblast and leukocyte types) on bone marrow and peripheral blood erythroid colony formation by cells from patients with various disorders of erythropoiesis was studied. Bone marrow or peripheral blood mononuclear cells were isolated and cultured in plasma clots with Epo, and benzidine-positive erythroid colonies counted after 7 to 14 days' incubation. Specimens included cells from patients with thalassemia, sickle cell anemia, secondary polycythemia, nutritional anemia, hemolytic anemia, refractory anemia, and normal controls. Results show that with the exception of nutritional anemia cells, erythroid colony formation by all specimens was significantly inhibited (84% to 100%) by 100 to 200 U of either interferon type per milliliter. Erythroid colony formation by nutritional anemia bone marrow cells ws inhibited only 30% to 40% by 200 U/ml fibroblast or leukocyte interferon, and 100 U/ml were ineffective. Sickle cell peripheral blood mononuclear cells and refractory anemia bone marrow demonstrated marked inhibition of colony formation (86% to 97%) with 50 U/ml fibroblast or leukocyte interferon. Inhibition of colony formation by sickle cell peripheral blood mononuclear cells was completely abolished by addition of anti-interferon. Colony formation by refractory anemia bone marrow was inhibited 46% to 51% by as little as 10 U/ml fibroblast or leukocyte interferon. This concentration of interferon was ineffective with cells from thalassemia, secondary polycythemia, nutritional anemia, hemolytic anemia, and controls. Mouse bone marrow colony formation was not suppressed by 200 U/ml leukocyte interferon. These results demonstrate that fibroblast or leukocyte interferons inhibit in vitro erythroid colony formation by human bone marrow or peripheral blood mononuclear cells, the effect is abolished by anti-interferon, and inhibition may be species-specific. These studies reveal that cells obtained from certan patients are particularly sensitive to the cytoxic effects of interferon, and it may be useful to monitor the erythropoietic state of the patient during interferon chemotherapy.     

Blood smear analysis in babesiosis, ehrlichiosis, relapsing fever, malaria, and Chagas disease

Blood smear analysis is especially useful for diagnosing five infectious diseases: babesiosis, ehrlichiosis, relapsing fever due to Borrelia infection, malaria, and American trypanosomiasis (Chagas disease). It should be performed in patients with persistent or recurring fever or in those who have traveled to the developing world or who have a history of tick exposure, especially if accompanied by hemolytic anemia, thrombocytopenia, or hepatosplenomegaly.