Clinical Characteristics and Treatment Outcomes of Patients With Autoimmune Hemolytic Anemia

April 11, 2021 updated by: Ehab A. Abdelmenam, Assiut University

Clinical Characteristics and Treatment Outcomes of Patients With Autoimmune Hemolytic Anemia in Assiut University Hospital

Autoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either warm reactive or cold reactive. The rate of hemolysis and the severity of the anemia may vary from mild to severe and life-threatening. Diagnosis is made in the laboratory by the findings of anemia, reticulocytosis, a positive Coombs test, and specific serologic tests. The prognosis is generally good but renal failure and death sometimes occur, especially in cases mediated by drugs.

Study Overview

Status

Completed

Conditions

Detailed Description

Hemolysis is the premature destruction of erythrocytes. A hemolytic anemia will develop if bone marrow activity cannot compensate for the erythrocyte loss. The severity of the anemia depends on whether the onset of hemolysis is gradual or abrupt and on the extent of erythrocyte destruction Hemolysis can be due to hereditary and acquired disorders. Acquired causes of hemolysis include immune disorders, Toxic chemicals and drugs, Antiviral agents and Physical damage .

Autoimmune hemolytic anemia (AIHA) exhibits two important characteristics: shortened red blood cell (RBC) survival and presence of autoantibodies directed toward autologous RBCs, usually demonstrated by a positive direct antiglobulin (Coombs) test (DAT). AIHA is classified by the temperature at which autoantibodies bind optimally to RBCs. In warm antibody AIHA, which constitutes about 80-90% of adult cases, hemolysis is mediated by antibodies which bind to RBCs at 37 °C (98.6 °F. In cryopathic hemolytic syndromes, cold-reactive autoantibodies exhibit affinity for RBCs optimally at temperatures below body temperature. In adults, most of the cold-reactive antibodies are agglutinins of the immunoglobulin M (IgM) isotype. In children, cold hemolysins (generally IgG) are most common. Unusual patients with mixed AIHA exhibit both cold-reactive and warm-reactive autoantibodies .

Each of these types of AIHA may be sub-classified based on the presence or absence of underlying diseases. In the absence of an underlying disease, the AIHA is termed primary or idiopathic. When AIHA occurs as a manifestation or complication of another disease, the term secondary AIHA is used. Chronic lymphocytic leukemia (CLL) and lymphomas account for about half of all secondary AIHA cases. Systemic lupus erythematosus (SLE) and other autoimmune diseases also account for a large number of secondary AIHA cases. Certain drugs also mediate immune injury to RBCs. Three mechanisms are recognized and two of these involve elements of autoimmunity, in which autoantibodies recognize and bind to specific RBC epitopes in concert with drugs or their metabolites or in which the autoantibody binds to RBC epitopes without the presence of drug

Diagnosis The signs and symptoms of AIHA are nonspecific and common to all types of anemia . The primary symptoms include exertional dyspnea, dyspnea at rest, varying degrees of fatigue, and signs and symptoms of the hyperdynamic state, such as bounding pulses, palpitations…etc

The laboratory findings of drug-induced AIHA may resemble those of warm antibody AIHA or a cryopathic hemolytic syndrome, depending on the mechanism of the autoantibody induction and the target antigen. A high index of suspicion on the part of the clinician and discussions with blood bank personnel can usually reveal the role of the drug.

complete blood count Peripheral blood smear : This test can detect an anemia, pancytopenia, and infections. Along with the differential count, a complete blood count can help diagnose hematologic malignancies and other hematological disorders. The platelet count usually is normal in most hemolytic anemias.

An increased reticulocyte count represents increased RBC production and is a criterion for hemolysis but is not specific for hemolysis. Thrombocytopenia associated with positive direct coombs test occur in evans syndrome .

Serum lactate dehydrogenase (LDH) study : Serum LDH elevation is a criterion for hemolysis, but is not specific

Serum haptoglobin : A low serum haptoglobin level is a criterion for moderate-to-severe hemolysis.

Indirect bilirubin : Unconjugated bilirubin is a criterion for hemolysis, but it is not specific , the level of indirect bilirubin usually is less than 3 mg/dL.

Direct antiglobulin test (DAT):

The DAT result is usually positive in autoimmune hemolytic anemia (AIHA), but it may occasionally be negative in this disorder. DAT-negative AIHAs have been reviewed. From 5-10% of all AIHAs are DAT negative. The polybrene test can detect DAT-negative AIHA.

Treatment all of the drugs used in the treatment of AIHA are largely or partly directed against the patient's natural immune system, which may, in turn, extensively derail the immune system due to treatment. .The first-line therapy for warm AIHA are corticosteroids, which are effective in 70-85% of patients and should be slowly tapered over a time period of 6-12 months. For refractory/relapsed cases, the current sequence of second-line therapy is splenectomy (effective approx. in 2 out of 3 cases but with a presumed cure rate of up to 20%), rituximab (effective in approx. 80-90% of cases), and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases. In coronary artery disease , rituximab is now recommended as first-line treatment

Study Type

Observational

Enrollment (Actual)

150

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Egypt
      • Assiut, Egypt
        • Assiut University Hospital

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Probability Sample

Study Population

patients above 18 years or more diagnosed AIHA at Assiut university hospital

Description

Inclusion Criteria:

patients diagnosed warm auto Immune hemolytic anemia primary or secondary(CLL, Lymphoma, rheumatoid arthritis

, SLE,Drug-induced )

Exclusion Criteria:

  1. All types of congenital hemolytic anemia
  2. Microangiopathic hemolytic anemia ( Thrombotic thrombocytopenic purpura (TTP) , Hemolytic-uremic syndrome (HUS) , Disseminated intravascular coagulation (DIC) )
  3. Alloimmune hemolytic anemia
  4. pregnancy

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort
warm autoimmune hemolytic anemia
corticosteroid either oral form or intravenous followed by oral

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Change from baseline in hemoglobin
Time Frame: three week from baseline
Efficacy endpoint assesment
three week from baseline
frequency of drug-related side effects
Time Frame: three week from base line
safety endpoint
three week from base line

Secondary Outcome Measures

Outcome Measure
Time Frame
Dose of steroid treatment
Time Frame: at 3 weeks, three months, six month, one year from baseline
at 3 weeks, three months, six month, one year from baseline

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Assiut University

Investigators

  • Study Director: Essam Abdel Monem El-beih, professor, Assiut University
  • Principal Investigator: wael ahmed abbas, professor, Assiut University

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

October 1, 2017

Primary Completion (Actual)

January 24, 2021

Study Completion (Actual)

January 24, 2021

Study Registration Dates

First Submitted

August 28, 2017

First Submitted That Met QC Criteria

August 30, 2017

First Posted (Actual)

August 31, 2017

Study Record Updates

Last Update Posted (Actual)

April 14, 2021

Last Update Submitted That Met QC Criteria

April 11, 2021

Last Verified

April 1, 2021

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • AIHA in assiut

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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