Thrombotic microangiopathy (TMA) is a group of highly heterogeneous, acute and severe clinicopathological syndromes, characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and ischemic injury of end organs. TMA has the characteristics of dangerous condition, multiple organ involvement and high mortality. Patients with severe TMA need to be admitted to intensive care unit (ICU) for organ function support therapy. Early and rapid evaluation, differential diagnosis, and timely and effective treatment are the key to improve the prognosis of TMA patients. Here, we review the pathophysiological changes, diagnosis differential diagnosis, and treatment of the severe TMA in adult.
Adult ; Humans ; Thrombotic Microangiopathies/therapy* ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Anemia, Hemolytic/therapy* ; Treatment Outcome ; Diagnosis, Differential

Background: Due to the high prevalence and incidence of leprosy in the Philippines, there is a continuing need to detect and document the occurrence of dapsone-induced hemolytic anemia. Objective: The aim of this study is to determine the incidence of dapsone-induced hemolytic anemia in non-glucose-6-phosphate dehydrogenase deficient leprosy patients receiving multidrug therapy (MDT) in Southern Philippines Medical Center. Methodology: This is a retrospective study through chart review of leprosy patients treated with MDT regimen at Southern Philippines Medical Center from January 2016 to December 2018. The demographic profile, clinical characteristics, hemoglobin and hematocrit concentrations before and after initiation of MDT, the presence of symptoms of anemia, and the occurrence of dapsone-induced hemolytic anemia in leprosy patients were collected. The main outcome measure for this study was the incidence rate of dapsone- induced hemolytic anemia. Statistical-based analysis were used for continuous and categorical data which were summarized using means and standard deviations, and frequencies and percentages, respectively. Results: There was a decrease in the mean hemoglobin and hematocrit levels noted in the majority of patients after initiation of MDT from baseline 143.46 g/dl and 0.44, respectively, to 94 g/dl and 0.28 on the third month of MDT. The incidence rate of dapsone-induced hemolytic anemia during the 3-year period was 20 cases per 100. Conclusion The relatively high incidence rate of dapsone-induced hemolytic anemia highlights the importance of frequent monitoring of hemoglobin and hematocrit concentrations in leprosy patients being treated with multidrug therapy.
Hansen&rsquo ; s disease ; leprosy ; Dapsone ; hemolytic anemia

Humans ; Adult ; Anemia, Hemolytic, Autoimmune/therapy* ; Anemia, Hemolytic

Sjögren's syndrome(SS)is a chronic autoimmune disease that affects exocrine glands, especially salivary and lacrimal glands. The main clinical manifestations are dry mouth and dry eyes, but also multi-organ and multi-system can be involved. Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature, resulting in skin microcirculation disturbance, or hemolytic anemia. Cold agglutinin disease is divided into two categories, primary cold agglutinin disease and secondary cold agglutinin disease. Primary cold agglutinin disease is characterized with cold agglutinin titer of 1 ∶4 000 or more and positive Coomb's test. However, the Coomb's test is not necessarily positive and the cold agglutinin titer is between 1 ∶32 and 1 ∶4 000 in secondary cold agglutinin disease. Here, we reported an elderly patient admitted to hospital due to fever. He was diagnosed with respiratory infection, but he showed incompletely response to the anti-infection treatment. Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies. Additionally, the patient complained that he had dry mouth and dry eyes for 1 year. Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren's syndrome. During the hospital stay, the blood clots were found in the anticoagulant tubes. Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin. In addition, further examination showed positive cold agglutination test with a titer of 1 ∶1 024, and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia. Furthermore, the patient developed cyanosis after ice incubating at the tip of the nose. Hence, the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments. Hence, the patient was diagnosed with SS combined with secondary CAD. SS combined CAD are rarely reported, and they are both autoimmune diseases. The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them. Cold agglutinin disease can lead to severe hemolytic anemia, even life-threatening. In clinical practice, timely recognizing and dealing with CAD might promote the prognosis of the patient.
Male ; Humans ; Aged ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Sjogren's Syndrome/diagnosis* ; Anemia, Hemolytic/complications* ; Dry Eye Syndromes/complications* ; Autoantibodies

OBJECTIVE: To investigate the clinical features and laboratory characteristics of primary autoimmune hemolytic anemia (AIHA) patients with negative results of direct antiglobulin test (DAT) by tube test but positive results by microcolumn gel assay, in order to provide references for the diagnosis of these patients. METHODS: 59 patients diagnosed with primary AIHA in our hospital from January 2015 to December 2020 were retrospectively analyzed. According to the results of tube test and microcolumn gel assay, the cases were divided into 3 groups, and the clinical and laboratory characteristics of each group were compared. RESULTS: The cases were grouped as follows: Group I, cases with negative results by both methods of DAT (n=5); Group II, cases with negative results by tube test but positive results by microcolumn gel assay (n=26); Group III, cases with positive results by both methods of DAT (n=28). There was no significant difference in age and sex between Group II and other groups, whereas the positive rate of anti-IgG + anti-C3d of Group II was lower than that in Group III (P=0.015). The main clinical manifestations of Group II were chest tightness, shortness of breath, fatigue, as well as yellow skin and sclera or dark urine, but the incidence rate of these symptoms was not significantly different from other groups. Anemia related indexes in Group II such as red blood cell (RBC) count and hemoglobin (Hb) were lower than the reference intervals, but there was no significant difference compared with other groups. Hemolysis related indexes in Group II such as reticulocyte (Ret) ratio, indirect bilirubin (IBIL), lactate dehydrogenase (LDH) and free-hemoglobin (F-Hb) were higher than the reference intervals, and the latter two items were signficantly higher than those in Group I (P=0.031 and P=0.036). Serum complement C3 and C4 in Group II were higher than those in Group III (P=0.010 and P=0.037). CONCLUSION Anemia severity of primary AIHA patients who were negative of DAT by tube test but positive by microcolumn gel assay was similar to those with negative or positive results by both DAT methods, but the mechanism and degree of complement system involved in hemolysis might be different. Results above may be helpful for laboratory diagnosis of this kind of patients.
Anemia, Hemolytic, Autoimmune/diagnosis* ; Bilirubin ; Complement C3 ; Coombs Test/methods* ; Erythrocytes ; Hemolysis ; Humans ; Lactate Dehydrogenases ; Negative Results ; Retrospective Studies

Objective: Determination of the prevalence of severe hematologic manifestations among Filipino patients with systemic lupus erythematosus (SLE) and analysis of any association with organ involvement and serology.

Methods: This cross-sectional study included SLE patients 19 years old and above seen at the UST Hospital from 2012 to 2017. Patients with severe hematologic manifestations (severe hemolytic anemia, severe thrombocytopenia, and Evans syndrome (ES)) were identified and their prevalence determined. Independent t-test was used to compare continuous variables. Categorical variables were measured using the chi-square test; odds ratios (OR) with their corresponding 95% confidence interval were calculated using the SPSS software version 21. This study has been approved by the Institutional Review Board.

Results: Of the 253 patients (238 females, 94.07%), the mean age at diagnosis was 27.04 (SD 9.96) years. Severe hematologic involvement was noted in 12.26% (n=31); severe hemolytic anemia was the most prevalent (14, 5.53%), followed by severe thrombocytopenia (13, 5.14%) and ES (4, 1.58%). Higher prevalence of major organ involvement was observed among patients who manifest with severe hematologic disease. Severe thrombocytopenia was more likely to have cardiac involvement (OR 7.39, 95% CI 1.90 to 28.81, p=0.004). A higher prevalence of negative baseline anti-dsDNA serology was seen among patients who developed ES.

Conclusion: Severe hematologic involvement was noted in 12.26% of Filipino SLE patients, with hemolytic anemia as the most frequently recorded. Severe thrombocytopenia was associated with cardiac involvement among these patients. Patients developing ES tend to have a negative anti-dsDNA serology.

A boy, aged 3 years and 8 months, had recurrent thrombocytopenia with hemolytic anemia for more than 3 years. The physical examination showed no enlargement of the liver, spleen, and lymph nodes or finger deformities. Laboratory results showed a negative result of the direct antiglobulin test, normal coagulation function, and increases in bilirubin, lactate dehydrogenase and reticulocytes. The results of von Willebrand factor-cleaving protease ADAMTS13 activity assay showed extreme deficiency, and antibody assay showed negative ADAMTS13 inhibitory autoantibodies. Next-generation sequence showed compound heterozygous mutation in the
ADAM Proteins/genetics* ; ADAMTS13 Protein ; Anemia, Hemolytic ; Autoantibodies ; Child ; Child, Preschool ; Humans ; Infant, Newborn ; Male ; Mutation ; Purpura, Thrombotic Thrombocytopenic

OBJECTIVE: To analyze one case of multiple myeloma (MM) initially presenting cold agglutinin syndrome (CAS), so as to improve clinical understanding and screening of this disease. METHODS: The clinical data, laboratory examination, bone marrow result, diagnosis and treatment of the patient were analyzed and summarized to provide ideas and clinical experience for the early diagnosis and treatment of CAS secondary to MM. RESULTS: The clinical manifestations of asthenia, hemolysis, jaundice and scattered livedo reticularis were caused by CAS secondary to MM, which was different from the general Raynaud's phenomenon. IgMκ type MM was definitely diagnosed according to the morphological features of bone marrow cells and immunofixation electrophoresis. After 3 courses of chemotherapy with BAD regimen and enhanced thermal support, anemia was corrected, M protein was decreased and the cold agglutinin phenomenon was significantly reduced. The evaluation of efficacy reached very good partial response. CONCLUSION There are very few MM patients with CAS as the initial presentation, so it is easy to misdiagnose. Early diagnosis and individual therapy are particularly important, which requires clinicians to observe and gain experience further.
Anemia, Hemolytic, Autoimmune/diagnosis* ; Cryoglobulins ; Early Diagnosis ; Humans ; Multiple Myeloma/diagnosis*

OBJECTIVE: To analyze the causes of positive irregular antibody screening test and incompatibility of cross matching in one patient with autoimmune hemolytic anemia complicated with neonatal hemolytic disease, and to accurately identify the type of antibodies in patients, and to select a reasonable strategy for blood transfusion. METHODS: One children was enrolled, blood group positive and reverse typing, Rh typing, direct anti-human globulin test, free test, dispersal test and cross matching test were carried out by test tube method and microcolumn gel card; irregular antibodies were identified by the reaction of DTT treatment and untreated panel cells with patients' plasma. RESULTS: The blood group of the patient was RhD positive B and irregular antibody screening positive, while the blood group of the mother was RhD positive O and irregular anti-screening negative, the result showed that the anti-LW detected in the plasma of the patient was autoantibody and ABO neonatal hemolytic disease (ABO-HDN) was present. Both O type RhD positive washing RBCs and B type RhD negative RBCs were transfused effectively. CONCLUSION Irregular antibodies in patients are anti-LW antibodies, and transfusion of homotype RhD negative suspended erythrocytes after the exclusion of ABO-HDN shows a better effect.
Anemia, Hemolytic, Autoimmune ; Autoantibodies ; Blood Group Incompatibility ; Blood Transfusion ; Erythroblastosis, Fetal ; Humans

OBJECTIVE: To evaluate the efficacy and safety of different types of red blood cell (RBC) transfusion and hormone therapy in patients with autoimmune hemolytic anemia (AIHA). METHODS: The clinical data and serological characteristics of 40 patients with AIHA treated in our hospital from 2014 to 2018 were collected and analyzed retrospectively. The efficacy and safety of different type of RBC transfusion and hormone therapy were evaluated according to the principle of minimally incompatible RBC transfusion after cross-matching. RESULTS: Among 40 patients with AIHA, the female cases were more than the male cases, the cases of secondary AIHA was more than cases of primary AIHA, and the warm autoantibodies were in the majority. 11 cases of AIHA underwent 26 times minimally incompatible red blood cell transfusions. The total effective rate was 46.2%, the partial efficiency was 23.1%, and total inefficiency was 30.8%. Among them, the same type of non-washing red blood cell group showed efficiency of 42.1%, partial effective rate of 21.1%, and inefficiency of 36.8%; the same type of washed red blood cell group showed efficiency of 57.1%, partial effective rate of 28.6%, and inefficiency of 14.3%. the infusion effects was not significanly different between the two groups, and no hemolytic transfusion reaction occurred. In the hormone-treated group, the complete remission rate was 15.2%, the partial remission rate was 63.6%, and the ineffective rate was 21.2%. Among them, the side effects appeared in 2 patients after using hormones. CONCLUSION When AIHA patients need blood transfusion, use the same type of non-washed red blood cells or homologous washed cells is relatively safe, and the difference in efficacy is not significant. The partial remission of patients received hormone therapy is much higher than that of red blood cell transfusion, but the side effects easily happen.
Anemia, Hemolytic, Autoimmune ; Autoantibodies ; Blood Transfusion ; Erythrocyte Transfusion ; Erythrocytes ; Female ; Humans ; Male ; Retrospective Studies