scholarly journals Warm antibody autoimmune hemolytic anemia

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 690-697 ◽  
Author(s):  
Theodosia A. Kalfa
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Standard Treatment ◽  
Treatment Options ◽  
Common Type ◽  
Therapeutic Approaches ◽  
Blood Disorder ◽  
Appropriate Treatment ◽  
Antiglobulin Test ◽  
Delays In Diagnosis

Abstract Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. AIHA caused by warm autoantibodies (w-AIHA), ie, antibodies that react with their antigens on the red blood cell optimally at 37°C, is the most common type, comprising ∼70% to 80% of all adult cases and ∼50% of pediatric cases. About half of the w-AIHA cases are called primary because no specific etiology can be found, whereas the rest are secondary to other recognizable underlying disorders. This review will focus on the postulated immunopathogenetic mechanisms in idiopathic and secondary w-AIHA and report on the rare cases of direct antiglobulin test–negative AIHA, which are even more likely to be fatal because of inherent characteristics of the causative antibodies, as well as because of delays in diagnosis and initiation of appropriate treatment. Then, the characteristics of w-AIHA associated with genetically defined immune dysregulation disorders and special considerations on its management will be discussed. Finally, the standard treatment options and newer therapeutic approaches for this chronic autoimmune blood disorder will be reviewed.

scholarly journals A New Murine Model of Primary Autoimmune Hemolytic Anemia (AIHA)

2021 ◽  
Vol 12 ◽  
Author(s):  
Flavia Dei Zotti ◽  
Annie Qiu ◽  
Francesca La Carpia ◽  
Chiara Moriconi ◽  
Krystalyn E. Hudson
Keyword(s):  
Hemolytic Anemia ◽  
Murine Model ◽  
Autoimmune Hemolytic Anemia ◽  
Molecular Mechanisms ◽  
Treatment Options ◽  
Management Strategies ◽  
White Pulp ◽  
Inflammatory Status ◽  
Humoral Tolerance ◽  
Made In

Loss of humoral tolerance to red blood cells (RBCs) can lead to autoimmune hemolytic anemia (AIHA), a severe, and sometimes fatal disease. Patients with AIHA present with pallor, fatigue, decreased hematocrit, and splenomegaly. While secondary AIHA is associated with lymphoproliferative disorders, infections, and more recently, as an adverse event secondary to cancer immunotherapy, the etiology of primary AIHA is unknown. Several therapeutic strategies are available; however, there are currently no licensed treatments for AIHA and few therapeutics offer treatment-free durable remission. Moreover, supportive care with RBC transfusions can be challenging as most autoantibodies are directed against ubiquitous RBC antigens; thus, virtually all RBC donor units are incompatible. Given the severity of AIHA and the lack of treatment options, understanding the cellular and molecular mechanisms that facilitate the breakdown in tolerance would provide insight into new therapeutics. Herein, we report a new murine model of primary AIHA that reflects the biology observed in patients with primary AIHA. Production of anti-erythrocyte autoantibodies correlated with sex and age, and led to RBC antigen modulation, complement fixation, and anemia, as determined by decreased hematocrit and hemoglobin values and increased reticulocytes in peripheral blood. Moreover, autoantibody-producing animals developed splenomegaly, with altered splenic architecture characterized by expanded white pulp areas and nearly diminished red pulp areas. Additional analysis suggested that compensatory extramedullary erythropoiesis occurred as there were increased frequencies of RBC progenitors detectable in the spleen. No significant correlations between AIHA onset and inflammatory status or microbiome were observed. To our knowledge, this is the first report of a murine model that replicates observations made in humans with idiopathic AIHA. Thus, this is a tractable murine model of AIHA that can serve as a platform to identify key cellular and molecular pathways that are compromised, thereby leading to autoantibody formation, as well as testing new therapeutics and management strategies.

scholarly journals Challenges in Treating Older Patients with Acute Myeloid Leukemia

2010 ◽  
Vol 2010 ◽  
pp. 1-11 ◽  
Author(s):  
Lagadinou D. Eleni ◽  
Zoumbos C. Nicholas ◽  
Spyridonidis Alexandros
Keyword(s):  
Acute Myeloid Leukemia ◽  
Clinical Judgment ◽  
Myeloid Leukemia ◽  
Treatment Options ◽  
Intensive Therapy ◽  
Treatment Decision ◽  
Social Background ◽  
Therapeutic Approaches ◽  
Appropriate Treatment ◽  
Acute Myeloid

Whereas in younger patients diagnosed with acute myeloid leukemia (AML) treatment is straightforward and the goal is cure, the optimal treatment decision for older adults remains highly controversial. Physicians need to determine whether palliation, “something” beyond palliation, intensive therapy, or an investigational therapy is the most appropriate treatment option. This requires understanding of the biology and risk profile of the AML, clinical judgment in evaluating the functional status of the patient, communication skills in understanding the patient's wishes and social background, and medical expertise in available therapies. The physician has to accurately inform the patient about (a) the unique biological considerations of his leukemia and his prognosis; (b) the risks and benefits of all available treatment options; (c) novel therapeutic approaches and how the patient can get access to these treatments. Last but not least, he has to recommend a treatment. This paper tries to discuss each of these issues.

Autoimmune hemolytic anemia: classification and therapeutic approaches

2008 ◽  
Vol 1 (2) ◽  
pp. 189-204 ◽  
Author(s):  
Pascal Sève ◽  
Pierre Philippe ◽  
Jean-François Dufour ◽  
Christiane Broussolle ◽  
Marc Michel
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Therapeutic Approaches

scholarly journals Clinical Patterns and Hematological Spectrum in Autoimmune Hemolytic Anemia

2010 ◽  
Vol 2 (01) ◽  
pp. 017-020 ◽  
Author(s):  
Vanamala Alwar ◽  
Shanthala Devi A M. ◽  
Sitalakshmi S. ◽  
Karuna R K.
Keyword(s):  
Connective Tissue ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Severe Anemia ◽  
Red Cell ◽  
Connective Tissue Disorders ◽  
Red Cell Membrane ◽  
Antiglobulin Test ◽  
Circulating Autoantibodies ◽  
Positive Direct

ABSTRACT Background: Autoimmune hemolytic anemia (AIHA) results from red cell destruction due to circulating autoantibodies against red cell membrane antigens. They are classified etiologically into primary and secondary AIHAs. A positive direct antiglobulin test (DAT) is the hallmark of diagnosis for AIHA. Methods and Results: One hundred and seventy-five AIHA cases diagnosed based on positive DAT were included in the study. The cases showed a female predilection (M: F = 1:2.2) and a peak incidence in the third decade. Forty cases were found to be due to primary AIHA, while a majority (n = 135) had AIHA secondary to other causes. The primary AIHA cases had severe anemia at presentation (65%) and more often showed a blood picture indicative of hemolysis (48%). Forty-five percent of primary AIHAs showed positivity for both DAT and indirect antiglobulin test (IAT). Connective tissue disorders were the most common associated etiology in secondary AIHA (n = 63). Conclusion: AIHAs have a female predilection and commonly present with symptoms of anemia. AIHA secondary to other diseases (especially connective tissue disorders) is more common. Primary AIHAs presented with severe anemia and laboratory evidence of marked hemolysis.

Direct antiglobulin test reactive with complement only in warm type autoimmune hemolytic anemia

2007 ◽  
Vol 0 (0) ◽  
pp. 071003000343002-???
Author(s):  
L. SHVIDEL ◽  
M. SHTALRID ◽  
A. DUEK ◽  
M. HARAN ◽  
A. BERREBI ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Direct Antiglobulin Test ◽  
Antiglobulin Test

scholarly journals Warm Autoimmune Hemolytic Anemia with a Direct Antiglobulin Test Positive for C3 and Negative for IgG: A Case Study and Analytical Literature Review of Incidence and Severity

2013 ◽  
Vol 6 ◽  
pp. CCRep.S11469 ◽  
Author(s):  
Amruth R. Palla ◽  
Farhad Khimani ◽  
Michael D. Craig
Keyword(s):  
Literature Review ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Interesting Case ◽  
Clinical Entity ◽  
Direct Antiglobulin Test ◽  
Complement C3 ◽  
Igg Antibodies ◽  
Antiglobulin Test

Polygenic IgG autoantibodies are implicated in majority of the cases of warm autoimmune hemolytic anemia (WAIHA). In some of these cases, complement (C3) proteins accompany the IgG antibodies. WAIHA mediated by C3 alone is relatively rare. We present an interesting case of WAIHA with a direct antiglobulin test (DAT) positive for C3 but negative for IgG in a 79-year-old woman and perform an analytical literature review of the incidence and severity of this clinical entity.

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