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

IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.
Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Autoimmune Diseases ; complications ; Biopsy ; Humans ; Immunoglobulin G ; Immunoglobulin G4-Related Disease ; complications ; diagnosis

OBJECTIVETo summarize the clinical features and therapy experience of a case of CD5 positive diffuse large B cell lymphoma (CD5+ DLBCL) with autoimmune hemolytic anemia (AIHA).

METHODSA 49-years old patient was investigated. The routine blood examination, bone marrow smear, Coombs test, serological test, chest CT, abdominal MR and immunochemistry etc were performed for this patient; and therapeutic effects of the chemotherapy regimen consisting of rituximab plus autologous hematopoietic stem cell transplantation (auto-HSCT) were observed.

RESULTSThe cervical lymphnode biopsy confirmed CD5+ DLBCL; the severe anemia, reticulocyte increase, Coombs test positive, and erythroid hyperplasia in bone marrow all suggested the occurence of autoimmune hemolytic anemia (AIHA). After plasma exchange, immune suppression using methylprednisolone, blood transfusion, one course of chemotherapy with "R-CHOP-E", the symptoms of AIHA in patient disappeared. After a continuous treatment for 3 courses of "R-CHOP-E", the bone marrow infiltration appeared, which was assessed as "PD", then the treatment was changed to the "R-ESHAP" for 4 courses, the patient was reassessed as "CR". The patient subsequently underwent auto-HSCT, followed up for 6 months, patientis still "CR".

CONCLUSIONThe status of the CD5+ DLBCL patient with AIHA is severe, and the prognosis is poor. The curative effect of the chemotherapy regimen with rituximab plus auto-HSCT for this patien is well.

Anemia, Hemolytic, Autoimmune ; therapy ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD5 Antigens ; metabolism ; Cisplatin ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Cytarabine ; therapeutic use ; Doxorubicin ; therapeutic use ; Etoposide ; therapeutic use ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Large B-Cell, Diffuse ; therapy ; Methylprednisolone ; therapeutic use ; Middle Aged ; Prednisone ; therapeutic use ; Rituximab ; therapeutic use ; Sentinel Lymph Node Biopsy ; Vincristine ; therapeutic use

Cold agglutinin disease is a kind of autoimmune hemolytic anemia, caused by cold agglutinin, serum autoantibodies activated at reduced body temperatures to produce red blood cell agglutination and hemolysis. In this paper we described a case of severe hemolytic anemia in a cold agglutinin disease patient treated with therapeutic plasma exchange. Therapeutic plasma exchanges were performed four times every other day. Over the same period, a total of 8 units of washed red blood cells were transfused. Then hemoglobin was increased from 4.0 g/dL to 7.8 g/dL. On the 12th hospital day hemoglobin level was decreased again to 4.2 g/dL and fludarabine chemotherapy was started on the 14th hospital day. The patient's symptoms were relieved and she was discharged on the 30th hospital day. As in this case, therapeutic plasma exchange could be considered as secondary therapy for temporary improvement of acute severe hemolytic anemia in cold agglutinin disease.
Agglutination ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Autoantibodies ; Body Temperature ; Drug Therapy ; Erythrocytes ; Hemolysis ; Humans ; Plasma Exchange*

Anemia, Hemolytic, Autoimmune ; drug therapy ; Drugs, Chinese Herbal ; Female ; Humans ; Middle Aged

OBJECTIVETo explore the safety and efficacy of lower dose of rituximab in the treatment of elderly autoimmune hemolytic anemia (AIHA).

METHODSFrom May 2008 to February 2013, a total of 37 patients with newly diagnosed elderly AIHA patients were enrolled in the study, including 25 cases treated with prednisone 1 mg · kg⁻¹ · d⁻¹ for 4 weeks and 12 cases ineligible for glucocorticoid receiving rituximab (100 mg/week for 4 times).

RESULTSOf the 25 patients with conventional glucocorticoid, 5 cases (20.0%) were complete remission (CR), 15 cases with partial remission (PR) and 5 cases without response. The overall response rate was 80.0%. Of the 12 cases with rituximab, 8 cases (66.7%) were CR, 3 cases with PR and 1 without response. The overall response rate was 91.7%. A significantly higher CR rate was seen in lower dose of rituximab, as compared to that in conventional glucocorticoid (P=0.038).

CONCLUSIONA lower dose of rituximab, with satisfactory safety and efficacy, was better than the conventional glucocorticoid in the treatment of elderly AIHA patients.

Aged ; Aged, 80 and over ; Anemia, Hemolytic, Autoimmune ; drug therapy ; Antibodies, Monoclonal, Murine-Derived ; administration & dosage ; adverse effects ; therapeutic use ; Female ; Humans ; Male ; Middle Aged ; Prednisone ; therapeutic use ; Rituximab ; Treatment Outcome

In order to screen the compatible red cells by using extracorporal hemolysis test for acute autoimmune hemolytic anemia (AIHA) patients who were difficult to be matched by automatic microcolumn gel indirect antiglobulin test. Twenty-six cases of AIHA were chosen as control group, to whom the same type of donor red blood cells were infused with the weakest blood agglutination; 12 cases of acute AIHA patients were chosen as test group, these patients were difficult to be matched by automatic microcolumn gel indirect antiglobulin test, and the donor red cells without hemolysis by extracoral hemolysis test were transfused for them. The results showed that compared with the control group,the effect of transfusion was better in test group (P < 0.01), with 2.26 U leukocyte-depleted erythrocyte suspension in average, whose hemoglobin, reticulocyte and total bilirubin levels were changed significantly compared with those before blood transfusion (P < 0.01) . It is concluded that the compatible red blood cells for the acute AIHA patients can be screened by the extracorporal hemolysis test, when it is difficult to screen by the automatic microcolumn gel indirect antiglobulin test.
Anemia, Hemolytic, Autoimmune ; therapy ; Blood Transfusion ; Coombs Test ; Erythrocyte Count ; Erythrocytes ; Hemolysis ; Humans ; Platelet Transfusion

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.
Acute Disease ; Adult ; Anemia, Hemolytic, Autoimmune/*complications/*diagnosis/drug therapy ; Antineoplastic Agents, Hormonal/therapeutic use ; Bone Marrow/pathology ; Female ; Hepatitis A/*complications/*diagnosis ; Humans ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Red-Cell Aplasia, Pure/*complications/*diagnosis/drug therapy ; Treatment Outcome ; Young Adult

BACKGROUNDAutoimmune hemolytic anemia (AIHA) is an uncommon complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) which has only been reported in a few cases. We here aimed to explore its mechanism.

METHODSWe retrospectively analyzed 296 patients who underwent allo-HSCT in our center from July 2010 to July 2012. Clinical manifestations were carefully reviewed and the response to currently available treatment approaches were evaluated. The survival and risk factors of AIHA patients after allo-HSCT were further analyzed.

RESULTSTwelve patients were diagnosed with AIHA at a median time of 100 days (15-720 days) after allo-HSCT. The incidence of AIHA after allo-HSCT was 4.1%. IgG antibody were detected in ten patients and IgM antibody in two patients. The two cold antibody AIHA patients had a better response to steroid corticoid only treatment and the ten warm antibody AIHA patients responded to corticosteroid treatment and adjustment of immunosuppressant therapy. Rituximab was shown to be effective for AIHA patients who failed conventional therapy. Survival analysis showed that the combination of AIHA in allo-HSCT patients hinted at poor survival. Cytomegalovirus (CMV) infection, graft-versus-host disease (GVHD) and histocompatibility leukocyte antigen (HLA) mismatch seemed to increase the risk of developing AIHA.

CONCLUSIONSPatients who develop AIHA after allo-HSCT have poor survival compared to non-AIHA patients. Possible risk factors of AIHA are CMV infection, GVHD, and HLA mismatch. Rituximab is likely to be the effective treatment choice for the refractory patients.

Adolescent ; Adrenal Cortex Hormones ; therapeutic use ; Adult ; Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; etiology ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Child ; Child, Preschool ; Female ; Graft vs Host Disease ; diagnosis ; drug therapy ; etiology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Rituximab ; Transplantation, Homologous ; adverse effects ; Young Adult

This study was aimed to analyze the serological characteristics, efficacy and safety of incompatible RBC transfusion in patients with autoimmune hemolytic anemia (AIHA). The patients with idiopathic or secondary AIHA were analyzed retrospectively, then the serological characteristics and the incidence of adverse transfusion reactions were investigated, and the efficacy and safety of incompatible RBC transfusion were evaluated according to the different autoantibody type and infused different RBC components. The results showed that out of 61 cases of AIHA, 21 cases were idiopathic, and 40 cases were secondary. 8 cases (13.1%) had IgM cold autoantibody, 50 cases (82.0%) had IgG warm autoantibody, and 3 cases (4.9%) had IgM and IgG autoantibodies simultaneously. There were 18 cases (29.5%) combined with alloantibodies. After the exclusion of alloantibodies interference, 113 incompatible RBC transfusions were performed for 36 patients with AIHA, total efficiency rate, total partial efficiency rate and total inefficiency rate were 56.6%, 15.1% and 28.3%, respectively. Incompatible RBC transfusions were divided into non-washed RBC group and washed RBC group. The efficiency rate, partial efficiency rate and inefficiency rate in non-washed RBC group were 57.6%, 13.0% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in washed RBC group were 53.6%, 21.4% and 25.0%, respectively. There was no significant difference of transfusion efficacy (P > 0.05) in two groups. Incompatible RBC transfusions were also divided into IgM cold autoantibody group and IgG warm autoantibody group. The efficiency rate, partial efficiency rate and inefficiency rate in IgM cold autoantibody group were 46.2%, 30.8% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in IgG warm autoantibody group were 56.7%, 13.4% and 29.9%, respectively. There was no significant difference of transfusion efficacy (P > 0.05 ) in two groups. Hemolytic transfusion reaction was not observed in all incompatible RBC transfusions. It is concluded that the same ABO type of non-washed RBC transfusion and O type washed RBC transfusion are all relatively safe for the AIHA patients with severe anemia after the exclusion of alloantibodies interference. There is no significant difference of transfusion efficacy in two groups. The same ABO type of non-washed RBC transfusion is more convenient and efficient than washed RBC transfusion, and excessive use of type O RBCs can also be avoided.
Adult ; Aged ; Aged, 80 and over ; Anemia, Hemolytic, Autoimmune ; diagnosis ; immunology ; therapy ; Blood Grouping and Crossmatching ; Erythrocyte Transfusion ; Female ; Humans ; Isoantibodies ; Male ; Middle Aged ; Treatment Outcome ; Young Adult