Autoimmune hemolytic anemia predominantly associated with IgA anti-E and anti-c

A patient with warm autoimmune hemolytic anemia (AIHA) due to predominance of immunoglobulin A (IgA) with an Rh specificity, considered to be the first case in Korea, is described. A 13-year-old male patient with severe hemolytic anemia showed a weak reactivity (1+) in the direct antiglobulin test (DAT) by using anti-IgG antiglobulin reagent. This finding, however, could not fully explain the patient's severe AIHA. When anti-IgA reagent was used for the DAT, strong reactivity (4+) was observed and free anti-E and anti-c autoantibodies were also detected by anti-IgA and anti-IgG reagents. The patient's hemoglobin began to rise with the administration of steroids. Because RBCs coated with multiple types of immunoglobulins are associated with more severe hemolysis than those only with IgG, the DATs using anti-IgA and other reagents are needed for the correct diagnosis when the result of DAT is not compatible with patient's clinical manifestations.     

Immune hemolytic anemia associated with probenecid

Upon hospital admission a patient was found to have severe anemia and a strongly positive direct antiglobulin test (DAT). The patient was taking probenecid periodically for gout. An antibody was detected in the patient's serum that only reacted with red blood cells (RBCs) when probenecid was added. Eluates from the patient's RBCs, with and without the presence of drug, were nonreactive. Upon the discontinuation of probenecid, the patient's hemoglobin level improved steadily. We believe this to be the first reported case of immune hemolytic anemia associated with probenecid.     

Autoimmune hemolytic anemia associated with benign ovarian cyst: a case report and review of literature

A 28-years-old female presented with severe autoimmune hemolytic anemia (AIHA), warm antibody mediated, and right side tubo-ovarian (TO) mass. There was sub-optimal response to immunosuppressive therapy (IST). Eight months later she underwent ovarian cystectomy which resulted in complete remission of AIHA without the need for IST. Pathological examination of the TO mass revealed benign ovarian cyst. Our case highlights the need to search for ovarian cyst/mass in females presenting with AIHA. Since immunosuppressive therapy is usually ineffectual in AIHA associated with ovarian cyst, it may be appropriate to contemplate surgery before embarking on IST in such cases.     

Autoimmune hemolytic anemia as a manifestation of T-suppressor-cell deficiency

This report describes two children in whom autoimmune hemolytic anemia was the initial clinical manifestation of an underlying T-cell deficiency. Further investigation revealed a profound deficiency of T suppressor cells in both children as detected by monoclonal T-cell antibody (OKT8) and/or functional assays. In vitro incubation of their lymphocytes with cultured thymus epithelium or thymic factors induced T suppressor cells. In vivo treatment with cultured thymus epithelium or calf thymosin fraction 5 resulted in increased T-suppressor-cell numbers and/or function in both and possibly decreased hemolytic activity in one. These results suggest that the autoimmune process in some patients with autoimmune hemolytic anemia is associated with T-suppressor-cell deficiency and that in vivo therapy with agents that modulate T-cell function may be of therapeutic value.     

Autoimmune hemolytic anemia with gel-based immunohematology tests: neural network analysis

In a previous report, we investigated the capability of commercially available immunohematology tests based on gel technology to add useful information for the diagnosis of autoimmune hemolytic anemia (AIHA). In this report, we analyzed the same casuistic to find useful information on the importance of different immunohematology tests for the AIHA diagnosis, but using the artificial neural network (ANN) analysis. We studied 588 samples with a positive direct antiglobulin test (DAT), of which 52 samples came from patients with AIHA. The samples were analyzed with the ANN using the multilayer perceptron with the backpropagation algorithm. Using the ANN in the observed data set, the predictive value for the presence of AIHAs was 94.7 %. The rate of DAT-positive cases that were not AIHA and that were correctly classified was 99.4 %. The receiver operating curve area for the model was 0.99. The independent variable importance analysis found that the gel centrifugation test anti-IgG titer was an important contributor to the network performance, but other variables such as the IgG subclasses can also be considered important. The use of the ANN permitted us to identify immunohematology tests that were “hidden” with the common statistical models used previously. This was the case for the IgG subclasses. However, it is very likely that the information given to the network from those tests is quantitative rather than qualitative.     

Autoimmune hemolytic anemia and a further example of autoanti-Kpb

A 65-year-old Caucasian man with myelodysplasia was admitted with autoimmune hemolytic anemia and a Hb of 5.6 g/dL. The patient's serum contained anti-K; the DAT on the patient's RBCs reacted 3+ with anti-IgG and 3+ with anti-C3d. K- RBC units were transfused, but there was no sustained increase in Hb level. The samples were referred to the reference laboratory of the National Blood Service. The DAT results remained the same, with anti-K detected only in the serum. An eluate prepared from the patient's DAT-positive RBCs revealed anti-Kp(b) specificity. This study reports an unusual case of autoanti-Kp(b), which is different from previously published cases in that no free anti-Kp(b) was detectable in the serum.     

Autoimmune hemolytic anemia in a patient with primary ovarian non-Hodgkin's lymphoma

The primary ovarian lymphoma is a rare disease with poor prognosis. The incidence of autoimmune hemolytic anemia in patients with non-Hodgkin's lymphoma is estimated at 3%. However, a substantial portion of the previously reported cases of ovarian lymphoma actually represented ovarian involvement by more diffuse lymphomatous process. If stringent criteria are used for case selection, true primary ovarian lymphoma usually carries a favorable prognosis. We present a primary malignant lymphoma of ovary accompanied by autoimmune hemolytic anemia in a 29-yr-old patient. After ablative surgery, the hemoglobin level and the reticulocyte count were normalized. One year following surgery and chemotherapy, the patient is alive and disease free.     

Autoimmune hemolytic anemia due to monoclonal IgM lambda anti-Tja (Anti-P+1+Pk)

A patient with autoimmune hemolytic anemia of the cold antibody type is described. The monoclonal autoantibody had mu heavy and lambda light chains and Tja blood group specificity. The antibody resulted in acute hemolysis responsive to steroid treatment and appeared simultaneously with an increase in CMV titer.     

Warm autoimmune hemolytic anemia associated with an IgM autoanti-Ge

A 28-year-old male with a prior history of Hodgkin's disease and a recent upper respiratory tract infection presented with autoimmune hemolytic anemia (AIHA). The patient's red blood cells (RBCs) were spontaneously agglutinated after room temperature and 37 degrees C washes. Dithiothreitol-treated RBCs reacted strongly with anti-C3 and were nonreactive with anti-IgG, -IgM, and -IgA; they reacted with anti-IgM (kappa light chains only) by flow cytometry. The patient's serum was nonreactive. An acid eluate was only weakly reactive, but a 56 degrees C heat eluate strongly agglutinated untreated RBCs (3+). Ficin-treated RBCs were nonreactive. En(a-) RBCs were strongly reactive, but Ge- RBCs were nonreactive. The anti-Ge in the eluate was IgM. The patient's untreated RBCs were shown, by flow cytometry, to be weakly Ge+. This is the first report of IgM-mediated warm AIHA associated with autoanti-Ge.     

Serous tumor of low malignant potential with early stromal invasion (serous LMP with microinvasion)

Eighteen examples of serous carcinoma arising in a background of a serous tumor of low malignant potential (SLMP with microinvasion) are reported. Nine of the 18 patients were less than 35 yr of age, and 5 were pregnant. In one patient, the microinvasive tumor was bilateral. The contralateral ovary had a serous tumor of low malignant potential (SLMP) in 9 additional women. Twelve patients presented with Stage I tumors, while 6 (33%) had tumors in Stages II and III. The tumors in all patients below the age of 30 were Stage I, and all three patients with Stage III tumors were older than 50. Two cell types were identified in all 18 tumors. The more dominant cell type was similar to those found in the typical SLMP tumors. The second cell type was larger with abundant eosinophilic cytoplasm, round nuclei, and prominent nucleoli; these were the cells that invaded the stroma of the papillary stalks in every case. Only one patient died of her disease, and she had Stage III tumor with massive ascites at presentation. All other patients are alive and well without evidence of disease from 2.5 to 5.5 yr after the diagnosis.     

Late recurrence (33 years) of a gastric epithelioid stromal tumor (leiomyoblastoma) with low malignant potential

A recurrence of a gastric epithelioid stromal tumor (leiomyoblastoma) 33 years after incomplete resection and radiation therapy is reported. The original tumor involved the posterior gastric wall and presented with intraperitoneal hemorrhage. A tumor recurrence was found attached to the stomach with invasion through the wall of the splenic flexure of the colon. The histologic features of the tumor had not significantly altered, except for a slight increase in mitotic rate. Neither the original tumor nor the recurrence exhibited smooth-muscle differentiation on ultrastructural or immunohistochemical examination, suggesting an origin from an undifferentiated or primitive mesenchymal cell. The term leiomyoblastoma, although established in the literature, should be reserved for epithelioid tumors in which myoid differentiation can be established. The long interval to recurrence supports the suggestion that all of these tumors have at least a low-grade malignant potential.