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*

A 34-year-old lady presenting with features of cold agglutinin disease during the course of systemic lupus erythematosus is described. Cold antibody titer was very high (1 in 4096) with specificity for 'I' antigen. Even though she had poor prognostic factors like high titer of cold antibodies with low thermal amplitude, she responded well to prednisolone.
Adult ; Anemia, Hemolytic, Autoimmune/immunology ; Anemia, Hemolytic, Autoimmune/etiology* ; Anemia, Hemolytic, Autoimmune/drug therapy ; Antibodies/analysis ; Case Report ; Female ; Human ; Lupus Erythematosus, Systemic/complications* ; Prednisolone/therapeutic use ; Prognosis

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

Adrenal Cortex Hormones ; therapeutic use ; Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Female ; Humans ; Infant ; Treatment Outcome

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

A pediatric patient with combined primary thrombocytopenic purpura and acquired hemolytic anemia (Evans syndrome), whose condition did not respond to treatment with prednisolone, has enjoyed long-term remission following a period of treatment with 6-Mercaptopurine.
6-Mercaptopurine/therapeutic use* ; Anemia, Hemolytic, Autoimmune/drug therapy* ; Child ; Human ; Male ; Purpura, Thrombocytopenic/drug therapy* ; Syndrome

OBJECTIVETo investigate the quantities of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia and the relationship between quantities of CD5+ B lymphocytes and clinical or laboratorial parameters.

METHODSQuantities of CD5+ B lymphocytes in the bone marrow of 14 patients with autoimmune hemolytic anemia (AIHA) or Evans syndrome, 22 immunorelated pancytopenia (IRP) patients, and 10 normal controls were assayed by flow cytometry. The correlation between their clinical or laboratorial parameters and CD5+ B lymphocytes was analyzed.

RESULTSThe quantity of CD5+ B lymphocytes of AIHA/Evans syndrome (34.64% +/- 19.81%) or IRP patients (35.81% +/- 16.83%) was significantly higher than that of normal controls (12.00% +/- 1.97%, P < 0.05). However, there was no significant difference between AIHA/Evans syndrome and IRP patients (P > 0.05). In all hemocytopenic patients, the quantity of bone marrow CD5+ B lymphocytes showed significantly negative correlation with serum complement C3 level (r = -0.416, P < 0.05). In the patients with AIHA/Evans syndrome, the quantity of bone marrow CD5+ B lymphocytes showed significantly positive correlation with serum indirect bilirubin level (r = 1.00, P < 0.05). In Evans syndrome patients, the quantity of CD5+ B lymphocytes in bone marrow showed significantly positive correlation with platelet-associated immunoglobulin G (r = 0.761, P < 0.05) and platelet-associated immunoglobulin M ( r = 0.925, P < 0.05). The quantity of CD5+ B lymphocytes in bone marrow of all hemocytopenic patients showed significantly negative correlation with treatment response (tau-b = -0.289, P < 0.05) , but had no correlation with colony forming unit-erythroid (r = -0.205, P > 0.05) or colony forming unit-granulocyte-macrophage colonies (r = -0.214, P > 0.05).

CONCLUSIONSThe quantity of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia significantly increases and is correlated with disease severity and clinical response, which suggest that CD5+ B lymphocytes might play an important role in the pathogenesis of autoimmune hemocytopenia.

Anemia, Hemolytic, Autoimmune ; drug therapy ; immunology ; Autoimmune Diseases ; drug therapy ; immunology ; B-Lymphocytes ; classification ; immunology ; Cyclosporine ; therapeutic use ; Drug Therapy, Combination ; Flow Cytometry ; Glucocorticoids ; therapeutic use ; Humans

Mixed type Evans syndrome is a very rare hematologic disease. Although mixed type Evans syndrome may initially respond well to steroids, this disease usually runs a chronic course with intermittent exacerbations. We describe here a 46-yr-old female with the steroid-refractory, mixed type Evans syndrome, and she had a prompt response to rituximab. She was diagnosed as having the mixed type Evans syndrome with the clinical features of symptomatic anemia, jaundice and thrombocytopenia. Prednisone therapy was commenced and her hemoglobin and platelet level returned to the normal. However, after 15 weeks, she relapsed with hemolytic anemia and thrombocytopenia. We started rituximab at the dose of 375 mg/m2 once weekly for a total of 4 doses, which was well-tolerated and this induced the normalization of hemoglobin, bilirubin and lactic dehydrogenase, and there was also a significant increase of the platelet count.
Treatment Outcome ; Syndrome ; Purpura, Thrombocytopenic, Idiopathic/*drug therapy ; Middle Aged ; Immunologic Factors/therapeutic use ; Humans ; Female ; Antibodies, Monoclonal/*therapeutic use ; Anemia, Hemolytic, Autoimmune/*drug therapy

Although Mycoplasma pneumonia is frequently observed in immunocompromized patient if, rarely results in acute respiratory distress syndrome (ARDS). The cold agglutinin is positive in 33-76% of patients with M. pneumonia infection. We experienced a case of ARDS due to mycoplasma pneumonia associated with cold agglutinin disease in primary CNS lymphoma. He was a 60-year old primary CNS lymphoma patient with mycoplasma pneumonia and cold agglutinin disease who rapidly progressed to ARDS after 1 cycle of chemotherapy. He completely recovered with roxithromycin, levofloxacin, and ventilator supports. After recovery 2nd cycle of combination chemotherapy and brain radiation therapy was given and, CNS lymphoma is in complete remission until now.
Anemia, Hemolytic, Autoimmune* ; Brain ; Drug Therapy ; Drug Therapy, Combination ; Humans ; Levofloxacin ; Lymphoma* ; Middle Aged ; Mycoplasma* ; Pneumonia ; Pneumonia, Mycoplasma* ; Respiratory Distress Syndrome, Adult* ; Roxithromycin ; Ventilators, Mechanical

Although Mycoplasma pneumonia is frequently observed in immunocompromized patient if, rarely results in acute respiratory distress syndrome (ARDS). The cold agglutinin is positive in 33-76% of patients with M. pneumonia infection. We experienced a case of ARDS due to mycoplasma pneumonia associated with cold agglutinin disease in primary CNS lymphoma. He was a 60-year old primary CNS lymphoma patient with mycoplasma pneumonia and cold agglutinin disease who rapidly progressed to ARDS after 1 cycle of chemotherapy. He completely recovered with roxithromycin, levofloxacin, and ventilator supports. After recovery 2nd cycle of combination chemotherapy and brain radiation therapy was given and, CNS lymphoma is in complete remission until now.
Anemia, Hemolytic, Autoimmune* ; Brain ; Drug Therapy ; Drug Therapy, Combination ; Humans ; Levofloxacin ; Lymphoma* ; Middle Aged ; Mycoplasma* ; Pneumonia ; Pneumonia, Mycoplasma* ; Respiratory Distress Syndrome, Adult* ; Roxithromycin ; Ventilators, Mechanical