scholarly journals Autoimmune hemolytic anemia associated with mesenteric teratoma

2017 ◽  
Vol 89 (1) ◽  
pp. 78-81
Author(s):  
K I Ntanishyan ◽  
K R Sabirov ◽  
O V Shcherbakova ◽  
D E Vybornykh ◽  
I A Shupletsova ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Corticosteroid Therapy ◽  
Autoimmune Hemolytic Anemia ◽  
Laparoscopic Splenectomy ◽  
Hypertensive Crisis ◽  
Hemoglobin Level ◽  
Sustained Remission ◽  
Hematological Response ◽  
Mesenteric Root ◽  
Temporary Response

The paper describes a case of autoimmune hemolytic anemia (AIHA) in a 27-year-old woman whose examination revealed mesenteric teratoma. AIHA was characterized by a hypertensive crisis and a temporary response to corticosteroid therapy that was complicated by the development of somatogenic psychosis and discontinued. A relapse of hemolysis developed 6 months later. The patient underwent laparoscopic splenectomy and removal of mesenteric root teratoma. Immediately after surgery, a hematological response was obtained as relief of hemolysis and restoration of a normal hemoglobin level. There is a sustained remission of AIHA for the next 16 months.

scholarly journals Method of spleen reduction in laparoscopic splenectomy in children with hereditary autoimmune hemolytic anemia

2020 ◽  
Vol 10 (4) ◽  
pp. 429-433
Author(s):  
Vyacheslav G. Svarich ◽  
Ilya M. Kagantsov ◽  
Violetta A. Svarich
Keyword(s):  
Surgical Treatment ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Laparoscopic Splenectomy ◽  
Surgical Intervention ◽  
Abdominal Cavity ◽  
Operation Time ◽  
Spleen Tissue ◽  
Surgical Department ◽  
Large Size

Purpose. This study aimed to improve the results of surgical treatment of children with hereditary autoimmune hemolytic anemia by laparoscopic splenectomy. Materials and methods. In the period from 1991 to 2020, a total 47 patients with hereditary autoimmune hemolytic anemia were treated in the surgical department of the Republican Childrens Clinical Hospital of Syktyvkar. Splenectomy was performed by the open method in 25 children, and laparoscopic method in 22 patients. Since 2019, the method of spleen reduction during laparoscopic splenectomy has been used in 3 patients when the large size of the mobilized spleen does not correspond to the size of the endoscopic container. Results. On average, surgical intervention using the above-described method of spleen reduction lasted for 19 2 min lesser than with laparoscopic splenectomy without the above method, due to the possibility of removing a significantly smaller volume of spleen tissue from the endoscopic sac outside the abdominal cavity. However, the most important achievement was the almost complete elimination of the risk of getting free fragments of a pathologically altered spleen with its possible replantation and recurrence of the clinic of autoimmune hemolytic anemia. The postoperative period was smooth, and all patients were discharged at their place of residence 7 days after the laparoscopic splenectomy. Intra-abdominal complications and relapses of the disease associated with the above-described method of operation did not occur in any patient within 612 months postoperative. Conclusion. The proposed method of spleen reduction during laparoscopic splenectomy made it possible to avoid relapses of the disease, reduce the operation time, as a result, improved the results of surgical treatment in children with hereditary autoimmune hemolytic anemia.

Rituximab Treatment for Autoinmune Hemolitic Diseases,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3168-3168
Author(s):  
Natalia Schutz ◽  
Jorge Arbelbide ◽  
Walter Skordo ◽  
Susana Viñuales ◽  
Elsa Nucifora ◽  
...  
Keyword(s):  
Partial Response ◽  
Median Time ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Response Rate ◽  
Complete Response ◽  
Infectious Complications ◽  
Hemoglobin Level ◽  
Published Data ◽  
Steroid Dependence

Abstract Abstract 3168 INTRODUCTION: Steroids are the first line treatment for Autoimmune Hemolytic Anemia (AHA) and Immune Thrombocytopenic Purpura (ITP). Second line treatments for patients with partial reponses or steroid dependence are controversial and have changed during the last decades. Several authors have proposed the use of Rituximab in these patients although published data are still sparse. OBJECTIVES: We reviewed the medical records of all the patients treated in our hospital with Rituximab in order to evaluate the response rate and security of this treatment. MATHERIALS AND METHODS: We included in the study all the patients older than 18 years with diagnoses of Immune Thrombocytopenia or Autoimmune Hemolytic Anemia treated with Rituximab. Patients with oncologic diseases that required specific chemotherapy apart from AHA or ITP were excluded. Rituximab was administered at a dose of 375mg/m3 weekly for 4 weeks. In patients with AHA we assessed the response according to the following criteria: complete response a hemoglobin level higher than 12 g/dl without hemolysis (normal bilirubin, LDH and haptoglobin) and partial response at least 2g/dl increase from the basal hemoglobin level with improvement in the hemolysis parameters. In patients with ITP we defined complete response as >100.000 platelets/mm3 and partial response >50.000 platelets/mm3. In both cases patients should be tapering steroids (less than 10mg/day of prednisone) and without transfusions. RESULTS: We performed 55 treatments with Rituximab in 37 pts., 19 AHA (12 cold hemolytic anemia) and 18 ITP. The median age was 60 years (26 – 86) and 31 pts. were female. Five patients had other autoimmune diseases (lupus, sjogren, autoimmune hepatitis), and six patients had an underlying onco-hematologyc disease (CLL, indolent lymphoma). All patients had been treated with steroids before and most of them had also received azathioprine, cyclophosphamide or gamaglobuline. The median of previous treatments was 3. Eight patients had undergone also splenectomy. The reason for the treatment was steroid dependence in 16 patients, partial response 6 patients and no response to previous treatment in 15 patients. The overall response rate was 79% for AHA and 94% for ITP, with 8pts (42%) and 13pts (72%) achieving a complete response and 7pts (37%) and 4pts (22%) achieving a partial response respectively. The median time to the complete response was 14 days for ITP and 28 days for AHA. The treatment was well tolerated with only one infusion related serious adverse event and one pulmonary thromboembolism during the period of treatment. Fourteen patients relapsed (8 AHA and 6 PTI). The response rate to Rituximab at relapsed for those patients that received a second or even third course of treatment were similar to the observed before. The median time of follow up was 50 months with and event free survival at 1 year of 34% (median 11,9 months) for AHA vs. 60% for PTI (median 38 months) (p 0,23). The overall survival at 5 years was 39% (median 53 months) vs. 86% (median not reached) (p 0,18) respectively. Seven patients with AHA and 2pts with ITP died, mostly of infectious complications. CONCLUSIONS: Rituximab is an effective treatment for ITP and AHA patients with no response to previous treatments or corticoid dependence. There were few serious adverse effects related to the treatment with Rituximab itself. The mortality rate was higher in patients with AHA although it wasn`t statistically significant. The main cause of death was related to infectious complications but most of the patients had a long history of immunosuppression treatment. Patients who relapsed and were treated again with rituximab had very good response rate. Disclosures: No relevant conflicts of interest to declare.

scholarly journals The Treatment of Autoimmune Hemolytic Anemia with 6-Mercaptopurine and Thioguanine

Blood ◽  
1962 ◽  
Vol 19 (4) ◽  
pp. 483-500 ◽  
Author(s):  
ROBERT S. SCHWARTZ ◽  
WILLIAM DAMESHEK
Keyword(s):  
Side Effects ◽  
Hemolytic Anemia ◽  
Corticosteroid Therapy ◽  
Autoimmune Hemolytic Anemia ◽  
Good Effect ◽  
Control Side ◽  
Degrees Of Severity

Abstract Fourteen patients with autoimmune hemolytic anemia were treated with either 6-mercaptopurine or thioguanine. Nine patients responded and five failed to improve. Eight patients developed side effects, either hematologic or gastrointestinal, of varying degrees of severity; in three the antimetabolite had to be discontinued, while in others adjustment of the dosage or the administration of antacids was sufficient to control side effects. Included in this series are nine patients who failed to respond adequately to corticosteroid therapy; four of these had a good effect from antimetabolite therapy. Although these results indicate that antimetabolites may reverse the course of autoimmune hemolytic anemia, the eventual role of these agents in the treatment of this disorder requires further study.

scholarly journals Prognostic significance of hemoglobin level and autoimmune hemolytic anemia in SARS-CoV-2 infection

2020 ◽  
Vol 100 (1) ◽  
pp. 37-43
Author(s):  
Abdulrahman A. Algassim ◽  
Assem A. Elghazaly ◽  
Abdulrahman S. Alnahdi ◽  
Owais M. Mohammed-Rahim ◽  
Abdulaziz G. Alanazi ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Prognostic Significance ◽  
Hemoglobin Level

Low-Dose Rituximab in Idiopathic Autoimmune Hemolytic Anemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4027-4027
Author(s):  
Wilma Barcellini ◽  
Francesco Zaja ◽  
Anna Zaninoni ◽  
Francesca Guia Imperiali ◽  
Marta Battista ◽  
...  
Keyword(s):  
Side Effects ◽  
Hemolytic Anemia ◽  
Corticosteroid Therapy ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Low Dose ◽  
Immunosuppressive Agents ◽  
Oral Prednisone ◽  
Fixed Dose ◽  
Line Therapy

Abstract Abstract 4027 Poster Board III-963 Background Conventional therapy of warm autoimmune hemolytic anemia (WAIHA) include administration of corticosteroids and immunosuppressive agents, or splenectomy, whereas no effective treatment exists for cold hemagglutinin disease (CHD). A substantial proportion of patients with WAIHA do not respond to or relapse after corticosteroid therapy and may experience clinically relevant side effects. Favorable responses to rituximab at standard doses (375 mg/m2 weekly for 4-6 courses) have been reported in both WAIHA and CHD, idiopathic or secondary, as well as in other autoimmune diseases, such as rheumatoid arthritis and primary immune thrombocytopenia. Recently, low dose (LD) rituximab (100 mg fixed dose weekly for 4 courses) has been proven effective in patients with autoimmune cytopenias, particularly immune thrombocytopenia. Aims To evaluate the safety, activity and the duration of the response of LD rituximab associated with standard oral prednisone (PDN) as first line therapy in newly diagnosed WAIHA and CHD, and as second line therapy in WAIHA relapsed after standard oral PDN. Methods In this single-arm prospective pilot study, LD-rituximab was administered at 100 mg fixed dose weekly on days +7, +14, +21, +28 along with standard oral PDN (1 mg/kg/die p.o. from day +1 to + 30, followed by quick tapering: 10 mg/week until 0.5/mg/kg/die, then 5 mg/week until stop). Complete and partial initial responses (iCR and iPR)) were defined as Hb ≥ 12 g/dL and ≥ 10 g/dL at month +2 from the beginning of therapy, respectively; sustained response (SR) was defined as Hb ≥ 10 g/dL at month +6 and +12, in the absence of any treatment. Results Twelve patients (6 female, 6 male; median age 49 yrs, range 28-65) were enrolled. A iCR and iPR were observed in 6 and 4 out of 12 patients, respectively; the median Hb level increased from 9.9 g/dL (range 6.1-12.3) at enrolment to 12.3 g/dL (range 9.5-14.9) at month +2. A SR at month +6 was observed in 7 out of 7 evaluable patients, and in the first 2 patients enrolled at month +12. No side effects or serious adverse events were observed. For 9 relapsed AIHA patients laboratory data (Hb, LDH, reticulocytes) and steroid administration were available before LD rituximab treatment: a lower cumulative dose (roughly 50%) of steroid was administered to patients during LD rituximab study compared with previous therapy, without significant differences in the general trend of Hb, LDH, and reticulocytes. Conclusions These preliminary results seem to indicate that the addition of LD rituximab to standard corticosteroid therapy is a feasible and active treatment in AIHA. Data on SR are intriguing, particularly regarding the possible steroid sparing effect of LD rituximab, but need to be confirmed in larger survey and after longer follow up. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Autoimmune Hemolytic Anemia and Nodular Lymphocyte-Predominant Hodgkin Lymphoma: A Rare Association

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Géraldine Salmeron ◽  
Thierry Jo Molina ◽  
Claire Fieschi ◽  
Anne-Marie Zagdanski ◽  
Pauline Brice ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Mediastinal Lymph Node ◽  
Hemoglobin Level ◽  
Lymphocytic Leukemia ◽  
Axillary Lymph Nodes ◽  
Axillary Lymph ◽  
Non Hodgkin Lymphoma ◽  
Positron Emission

Autoimmune hemolytic anemia (AIHA) has been associated with chronic lymphocytic leukemia, non-Hodgkin lymphoma, and classical Hodgkin lymphoma, but to the best of our knowledge, the association of AIHA and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) has not been reported previously. A 20-year-old woman presented with conjunctival jaundice, fever, asthenia, and hemoglobin 9.2 g/dL revealing IgG-mediated warm antibody AIHA. Computed tomography (CT) scan and positron-emission tomography (PET) scan showed mediastinal and axillary lymph nodes with increased [18F]-fluorodeoxyglucose uptake. A mediastinal lymph node was biopsied during mediastinoscopy, and NLPHL was diagnosed by an expert hematopathologist. The hemoglobin level declined to 4.6 g/dL. The treatment consisted of four 28-day cycles of R-ABVD (rituximab 375 mg/m2IV, adriamycin 25 mg/m2IV, bleomycin 10 mg/m2IV, vinblastine 6 mg/m2IV, and dacarbazine 375 mg/m2IV, each on days 1 and 15). Prednisone was progressively tapered over 10 weeks. After the first chemotherapy cycle, the hemoglobin level rose to 12 g/dL. After the four cycles, PET and CT scans showed complete remission (CR). At the last followup (4 years), AIHA and NLPHL were in sustained CR.

A Rare Case of Autoimmune Hemolytic Anemia Caused by Warm-reacting IgM-class Antibodies Associated with Mycoplasma Pneumoniae Infection

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S166-S166
Author(s):  
M Abdelmonem ◽  
H Wasim ◽  
M Abdelmonem
Keyword(s):  
Hemolytic Anemia ◽  
Mycoplasma Pneumoniae ◽  
Autoimmune Hemolytic Anemia ◽  
Hemoglobin Level ◽  
Cytotoxic Agents ◽  
Cold Agglutinin ◽  
Packed Red Blood Cells ◽  
Thermal Amplitude ◽  
History Of

Abstract Introduction/Objective Warm autoantibodies are usually IgG-class and/or IgA-class immunoglobulins. may be classified as agglutinins or hemolysins, which may be incomplete or complete, depending on in vitro serology; they almost always bind complement. Autoimmune hemolytic anemia with only warm IgM autoantibodies is extremely rare. Methods A 91-year-old Caucasian male with hypertension presented with non-productive cough for two weeks, associated with shortness of breath and fatigue. He has a paste history of medical history of high degree AV block with sick sinus syndrome and bradycardia. Results He had blood pressure of 111/53 and heart rate of 50. Laboratory investigations showed leukocytes count of 7, Hemoglobin level of 5.6 g/dl, hematocrit level of 15.5%, platelets count of 267, total bilirubin of 5.6 mg/dL, direct bilirubin of 0.7 mg/dL, and lactate dehydrogenase (LDH) of 372 U/L and IgM titer for Mycoplasma pneumoniae was 1493 units/mL (Ref < 770). He was transfused four units of packed red blood cells as emergency release due to a positive antibody screen. His hemoglobin level increased from 5.6 g/dL to 7.2 g/dL then it decreased from 8.2 g/dL to 5.6 g/dL the next two days. Direct antiglobulin test was 3+ positive for C3d and Negative for IgG. He finished a 7-day course of antibiotic treatment. The results the Cold-agglutinin titer and thermal amplitude test were suggestive of an IgM warm autoantibody and a cold agglutinin of a moderate titer. The results exclude a diagnosis of cold agglutinin syndrome. Conclusion Mycoplasma pneumonia is mainly associated with cold agglutinin syndrome but it is very rare to be associated with WAIHA. WAIHA with a DAT positive for C3 only is relatively rare with an incidence ranging between 6% and 13% and can have a clinical picture ranging from mild to severe anemia. Severe symptomatic hemolysis can be treated with Rituximab, cytotoxic agents or plasmapheresis.

scholarly journals Autoimmune Hemolytic Anemia Induced By Kangaroo Essence Pills (Report of Two Cases)

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4881-4881
Author(s):  
Yuesheng Meng ◽  
Zengmei An
Keyword(s):  
Red Blood Cells ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Blood Cells ◽  
Reticulocyte Count ◽  
Conflicts Of Interest ◽  
Hemoglobin Level ◽  
Cordyceps Sinensis ◽  
Direct Bilirubin ◽  
Total Serum

Abstract The autoimmune hemolytic anemia (AIHA) is characterized by the autoimmune destruction and shortened life span of red blood cells. The kangaroo essence pills (KEP)are marketed worldwide as an aphrodisiac agent. They may contain muscles and genital tissues of Australian kangaroo as the key active components. We report here two cases of autoimmune hemolytic anemia induced by the KEP. Patient One: A 68 years old female presented with malaise, dizziness, jaundice and deep-colored urine for three weeks. Before this, she had taken three brand names of KEP for several weeks. An anemia was diagnosed in a local hospital but the underlying cause was obscure. Treatment with iron, folic acid and mecobalamin was ineffective. Laboratory analysis revealed anemia with a hemoglobin level of 34.4g/L, reticulocyte count of 3.0%, elevated white blood cell count of 22.7×109/L with neutrophiles of 81.4%, and normal platelet count (98.4×109/L). The urine was positive for occult blood, proteinuria (+++), red blood cells (11-15/HP) and bilirubin (++). Total serum bilirubin was 93.3 umol/L and direct bilirubin 69.7umol/L. Both of direct and indirect antiglobulin tests (Coombs' tests) were positive. Cold agglutinins of IgM were negative. Ham’s test was negative. Activity of Glucose-6-phosphate dehydrogenase was normal. Bone marrow morphology showed erythroid hypercellularity, with a predominance of polychromatic normoblasts and orthochromatic normoblasts and abnormality of double-phases. A diagnosis of warm antibody autoimmune hemolytic anemia was made. Intravenous methylprednisolone with a daily dose of 80 mg was administered immediately in addition to transfusion of saline-washed red blood cells. Due to the severity of anemia, three regimens of daily plasmapheresis were given. Clinical conditions were improved rapidly. Two weeks later, the hemoglobin level was returned to normal range and she was discharged. Follow-up for one year proved no evidence of relapse. The patient’s history had no record of allergy and other health problems. The KEP was the only possible cause to induce hemolysis. Besides kangaroo tissues, some KEP may have other addictives such as ginseng, Chinese wolfberry, deers’ sinew, snowdeer’s penis, yak penis, wild donkey penis, cordyceps sinensis, and so on. It is yet known which ingredient, or even some impurities, were the prime culprit in this case. Patient Two: A 25 year old young woman presented with pale and dizziness for a week. CBC test showed decreased hemoglobin level 76.5g/L, reticulocyte count of 4.5%, increased direct bilirubin 48.2 umol/L, and a positive Coombs' test. Two weeks ago she had tried a brand name of KEP as analeptic agent. She was diagnosed with autoimmune hemolytic anemia and immediately cured with oral methylprednisolone. To our knowledge, the AIHA induced directly by “kangaroo essence pills” has yet been reported. It is suggested that caution should be made to take aphrodisiacs of unknown ingredients. Disclosures No relevant conflicts of interest to declare.

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