OBJECTIVE: To analyze the clinical features and laboratory characteristics of patients with cold agglutinin disease (CAD)/cold agglutinin syndrome (CAS) who were positive for acidified-serum lysis test (Ham test), and to compare them with Ham test negative CAD/CAS patients and paroxysmal nocturnal hemoglobinuria (PNH) patients, in order to provide references for the differential diagnosis of these diseases. METHODS: 53 patients diagnosed with CAD/CAS and 67 patients diagnosed with classic PNH in our hospital from January 2015 to December 2020 were retrospectively analyzed. The patients were grouped according to clinical diagnosis and results of cold agglutinin test (CAT), direct antiglobulin test (DAT), Ham test and PNH clone detection. The clinical and laboratory characteristics of each group were compared. RESULTS: The patients were grouped as follows: Ham^- CAD/CAS group, CAD/CAS patients negative for Ham test (n=36); Ham⁺ CAD/CAS group, CAD/CAS patients positive for Ham test (n=17); classic PNH group (n=67). Compared with the classic PNH group, the Ham⁺ CAD/CAS group had a higher median age (P =0.024), weaker positivity of Ham test, higher positive rates of CAT and DAT, and lower positive rate of PNH clone detection (all P <0.001). The proportions of patients with splenomegaly and cyanosis in Ham⁺ CAD/CAS group were significantly higher than those in classic PNH group (P =0.002 and P <0.001). Ham⁺ CAD/CAS group displayed lower red blood cell count (RBC) and lactate dehydrogenase (LDH) level (P =0.007 and P <0.001), and higher mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), and indirect bilirubin (IBIL) level (P =0.003, P =0.004 and P =0.006) than those in classic PNH group. The levels of serum complement C3 and C4 in Ham⁺ CAD/CAS group were lower than those in classic PNH group (P =0.001 and P <0.001). The positive rate of urinary occult blood in Ham⁺ CAD/CAS group was lower than that in classic PNH group (P =0.010). The clinical and laboratory characteristics of Ham⁺ CAD/CAS group were similar to those of Ham^- CAD/CAS group, except for median age, hemoglobin (Hb), MCHC, mean corpuscular volume (MCV), reticulocyte ratio (Ret), Ham test results, DAT positive types, and proportion of splenomegaly. CONCLUSION Some clinical features and laboratory indicators of CAD/CAS patients with positive results of Ham test are different from those of classic PNH patients, but relatively similar to those of CAD/CAS patients with negative results of Ham test. These results may provide a reference for differential diagnosis of related diseases.
Humans ; Anemia, Hemolytic, Autoimmune/blood* ; Retrospective Studies ; Hemoglobinuria, Paroxysmal/diagnosis* ; Female ; Male ; Coombs Test ; Diagnosis, Differential ; Middle Aged ; Adult

OBJECTIVE: To investigate the efficacy and safety of zanubrutinib in the treatment of autoimmune cytopenia (AIC) secondary to indolent B-cell lymphoma (iBCL). METHODS: A total of 23 patients with iBCL complicated with AIC who were admitted to our hospital from December 2019 to September 2023 were selected as the research subjects. All patients were administered zanubrutinib 160 mg, twice daily, and continued oral administration. The objective response rate (ORR) of AIC, the therapeutic effect on lymphoma, and the incidence of adverse reactions were observed. RESULTS: After a median follow-up of 20 (5 to 48) months, the median duration of response was 9 (interquartile range [IQR] 5-24)months. AICA efficacy assessment showed that there were 10 cases of complete remission (CR), 9 cases of partial remission (PR), and 4 cases of no response (NR), and the ORR was 82.6% (19/23) (95%CI : 61.2-95.0). Among them, for the 14 patients with autoimmune hemolytic anemia (AIHA), 7 achieved CR, 5 had PR, and 2 had NR. For the 4 patients with immune thrombocytopenia (ITP), 1 reached CR, 2 had PR, and 1 had NR. Regarding the 5 patients with Evans syndrome (ES), 2 achieved CR, 2 had PR, and 1 had NR. The assessment of lymphoma efficacy showed that there were 10 cases of CR , 7 cases of PR , 6 cases of stable disease (SD), and no progressive cases, with an ORR of 73.9% (17/23) (95%CI : 51.6-89.8). The main adverse reactions during the treatment were infection, hemorrhage, neutropenia, elevated lymphocyte count, rash, and anemia. Most of these adverse reactions were grade 1-2 and tolerable. No arrhythmia and hypertension occurred, and no deaths due to adverse reactions. CONCLUSION Zanubrutinib is effective and safe for AIC secondary to iBCL.
Humans ; Pyrazoles/therapeutic use* ; Lymphoma, B-Cell/complications* ; Pyrimidines/therapeutic use* ; Piperidines/therapeutic use* ; Female ; Male ; Anemia, Hemolytic, Autoimmune/etiology* ; Thrombocytopenia/etiology* ; Middle Aged ; Purpura, Thrombocytopenic, Idiopathic/etiology* ; Treatment Outcome ; Adult ; Aged ; Cytopenia

OBJECTIVE: To explore serological detection and blood transfusion strategies of mimicking antibodies, so as to provide appropriate transfusion strategies. METHODS: Detailed serological tests, including ABO blood group, Rh typing, antibody specificity, etc,were performed on two patients with autoimmune hemolytic anemia(AIHA). Meanwhile, the references about blood transfusion from mimicking antibody patients published from 1977 to 2024 in China and abroad were retrospectively summarized and analyzed. RESULTS: The patient 1 blood type was AB,CCDee and the antibody is mimicking anti-e, transfusion the e-negative red blood cells (RBCs) was effective. After two transfusions of e-RBCs, hemoglobin levels significantly increased from 48 g/L to 91 g/L, with complete resolution of hemolytic symptoms. The patient 2 blood type was O,CcDee, and the antibody was mimicking anti-c, the patient was diagnosed with AIHA and treated with hormone. No blood products were transfused during hospitalization, and his hemolysis was relieved. CONCLUSION Strictly grasping the indication of blood transfusion, blood transfusion should not be performed in the unnecessary conditions, and the corresponding antigen-negative RBC should be screened for transfusion in the necessay conditions.
Humans ; Blood Transfusion ; Anemia, Hemolytic, Autoimmune/therapy* ; ABO Blood-Group System ; Retrospective Studies ; Antibodies ; Male ; Blood Grouping and Crossmatching

Objective To investigate the clinical characteristics,treatment responses,and prognosis of patients with idiopathic multicentric Castleman disease(iMCD)complicated by autoimmune hemolytic anemia(AIHA). Methods The patients diagnosed with iMCD in Peking Union Medical College Hospital from January 2010 to December 2023 and having complete baseline blood routine data were retrospectively enrolled in the study.The iMCD patients were further assigned into AIHA and non-AIHA groups based on baseline laboratory examinations,and the clinical characteristics and prognosis were compared between the two groups. Results A total of 341 patients with iMCD were enrolled in this study,including 277(81.2%)exhibiting anemia at baseline.Five(1.8%)patients were identified as having iMCD-AIHA,all of whom were iMCD-not otherwise specified type complicated by warm antibody-type AIHA,and two of them were simultaneously diagnosed with Evans syndrome.The timing relationship between the first onset of AIHA and iMCD diagnosis varied,with 2 patients experiencing their first hemolytic episode prior to the diagnosis of iMCD.In terms of treatment,the therapy targeting iMCD was effective in alleviating AIHA.The AIHA group had a poorer prognosis(HR=4.61,95% CI=1.08-19.80,P=0.040)and a lower 5-year survival rate(90% vs.60%,P=0.024)than the non-AIHA group.Conclusions iMCD-AIHA is clinically rare,and AIHA can occur at different stages of iMCD.Although the primary treatment principle remains targeting iMCD,great attention should be paid to the exacerbation of immune dysfunction caused by AIHA and the possible adverse prognosis it may bring.
Humans ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Castleman Disease/diagnosis* ; Retrospective Studies ; Male ; Female ; Prognosis ; Adult ; Middle Aged ; Aged ; Young Adult ; Adolescent

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

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

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

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.