Medicine Marrow Pg 91-100 (Hematology)
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Questions and Answers

Which cells are characteristic of Warm Autoimmune Hemolytic Anemia?

  • Schistocytes (correct)
  • Leukocytes
  • Spherocytes
  • Erythrocytes

    • Cold Autoimmune Hemolytic Anemia is associated with C3 activation.

    True

    What is the ideal but not preferred treatment for relapses in autoimmune hemolytic anemia?

    Splenectomy

    The stain used for reticulocytosis is a ______ stain.

    <p>supravital</p> Signup and view all the answers

    Match the following conditions with their common associations:

    <p>Evans Syndrome = Warm autoimmune hemolytic anemia + thrombocytopenia Hereditary spherocytosis = Uniform spherocytes Cold agglutinin disease = C3 activation G6PD deficiency = Spherocytes</p> Signup and view all the answers

    Which of the following infections is NOT associated with non-immune mediated hemolytic anemia?

    <p>Gerardia</p> Signup and view all the answers

    Paroxysmal nocturnal hemoglobinuria is an inherited condition caused by mutations in the PIGA gene.

    <p>False</p> Signup and view all the answers

    What are the two main complement regulatory proteins affected due to the PIGA gene mutation?

    <p>CD55 and CD59</p> Signup and view all the answers

    The PIGA gene is located on the ______ chromosome.

    <p>X</p> Signup and view all the answers

    Match the following terms with their descriptions:

    <p>GPI anchor protein = Anchors complement regulatory proteins onto RBC surface Dapsone = Drug causing hemolytic anemia Chronic intravascular hemolysis = A characteristic of paroxysmal nocturnal hemoglobinuria PIGA gene mutation = Causes acquired hemolysis in PNH</p> Signup and view all the answers

    Which type of antibody is associated with Warm antibody autoimmune hemolytic anemia?

    <p>IgG</p> Signup and view all the answers

    The Indirect Coombs Test is more reliable than the Direct Coombs Test.

    <p>False</p> Signup and view all the answers

    What is the primary purpose of the Coombs test?

    <p>To differentiate between immune and non-immune hemolytic anemias.</p> Signup and view all the answers

    Paroxysmal cold hemoglobinuria is associated with the _____ antibody.

    <p>IgG</p> Signup and view all the answers

    Match the type of Coombs test with its description:

    <p>Direct Coombs Test = Detects antibodies on the surface of RBCs Indirect Coombs Test = Detects antibodies in circulation Positive Result = Indicates immune hemolytic anemia Negative Result = Suggests non-immune hemolytic anemia</p> Signup and view all the answers

    Which condition is most important in causing thrombotic microangiopathy (TMA)?

    <p>HUS</p> Signup and view all the answers

    Thrombotic thrombocytopenic purpura (TTP) primarily affects the renal capillaries.

    <p>False</p> Signup and view all the answers

    What is the primary site affected in Hemolytic Uremic Syndrome (HUS)?

    <p>Renal capillaries</p> Signup and view all the answers

    The triad of symptoms for Thrombotic Thrombocytopenic Purpura (TTP) includes microangiopathic hemolytic anemia, thrombocytopenia, and _____ .

    <p>renal failure</p> Signup and view all the answers

    Match the following features with HUS or TTP:

    <p>Renal failure = HUS Small vessel stroke = TTP Thrombocytopenia = Both Fever = Both</p> Signup and view all the answers

    What is the antigen involved in Paroxysmal Cold Hemoglobinuria?

    <p>P-antigen</p> Signup and view all the answers

    The Donath-Landsteiner test is used to investigate drug-induced hemolysis.

    <p>False</p> Signup and view all the answers

    Name one drug commonly associated with autoantibody-mediated hemolysis.

    <p>Methyldopa</p> Signup and view all the answers

    Paroxysmal Cold Hemoglobinuria induces hemolysis at ______ degrees Celsius.

    <p>37</p> Signup and view all the answers

    Match the following drugs to their corresponding hemolysis mechanism:

    <p>Methyldopa = Autoantibody-mediated Quinidine = Immune complex-mediated High dose penicillin = Adsorption Rifampicin = Immune complex-mediated</p> Signup and view all the answers

    Which immunoglobulin is primarily associated with Warm Autoimmune Hemolytic Anemia?

    <p>IgG</p> Signup and view all the answers

    Cold Autoimmune Hemolytic Anemia is characterized by IgG immunoglobulin.

    <p>False</p> Signup and view all the answers

    What temperature range is associated with Cold Autoimmune Hemolytic Anemia?

    <p>0-4°C</p> Signup and view all the answers

    The primary immunoglobulin in Cold Autoimmune Hemolytic Anemia is ______.

    <p>IgM</p> Signup and view all the answers

    Match the following autoimmune hemolytic anemia types with their characteristics:

    <p>Warm Autoimmune Hemolytic Anemia = IgG and 37°C Cold Autoimmune Hemolytic Anemia = IgM and 0-4°C Immunoglobulin = Antibodies involved in hemolytic anemia Etiology = Causes of autoimmune conditions</p> Signup and view all the answers

    What percentage of PNH III RBC's is considered significant in flow cytometry?

    <blockquote> <p>50%</p> </blockquote> Signup and view all the answers

    The Hams test is commonly used in the investigation of non-immune mediated hemolytic anemia.

    <p>False</p> Signup and view all the answers

    What is the preferred treatment for hemoglobinuria and thrombosis in this condition?

    <p>Ravulizumab</p> Signup and view all the answers

    In urine analysis, myoglobin can be detected when the urine is initially __________.

    <p>high colored</p> Signup and view all the answers

    Match the test or treatment with its purpose:

    <p>Flow cytometry = Detects PNH III RBC's percentage Coombs test = Identifies immune hemolysis Allogenic hematopoietic stem cell transplant = Address bone marrow involvement Meningococcal vaccine = Prevent infections post-treatment</p> Signup and view all the answers

    Which feature is associated with extravascular hemolysis?

    <p>Moderate splenomegaly</p> Signup and view all the answers

    Intravascular hemolysis is associated with increased urine hemoglobin.

    <p>True</p> Signup and view all the answers

    What condition is characterized by unconjugated hyperbilirubinemia as a result of hemolysis?

    <p>Extravascular hemolysis</p> Signup and view all the answers

    The site of extravascular hemolysis primarily occurs in the _____

    <p>spleen</p> Signup and view all the answers

    Match the parameters with their associated types of hemolysis:

    <p>Plasma Hb increase = Intravascular Hemolysis Splenomegaly = Extravascular Hemolysis Urine hemosiderin increase = Intravascular Hemolysis Tissue iron increase = Extravascular Hemolysis</p> Signup and view all the answers

    What primarily happens during cold autoimmune hemolytic anemia?

    <p>RBC agglutination in the extremities with IgM.</p> Signup and view all the answers

    Warm autoimmune hemolytic anemia primarily causes intravascular hemolysis.

    <p>False</p> Signup and view all the answers

    Name one clinical feature commonly seen in warm autoimmune hemolytic anemia.

    <p>Spherocytosis</p> Signup and view all the answers

    In cold autoimmune hemolytic anemia, _____ cells recognize the agglutinated RBCs.

    <p>Kupffer</p> Signup and view all the answers

    Match the following clinical features with the type of autoimmune hemolytic anemia:

    <p>Anemia, spherocytosis, jaundice = Warm autoimmune hemolytic anemia Acrocyanosis = Cold autoimmune hemolytic anemia Splenomegaly = Warm autoimmune hemolytic anemia Agglutinated RBC = Cold autoimmune hemolytic anemia</p> Signup and view all the answers

    What is the most common type of thrombosis associated with paroxysmal nocturnal hemoglobinuria (PNH)?

    <p>Hepatic venous thrombosis</p> Signup and view all the answers

    Aplastic anemia does not have any link to paroxysmal nocturnal hemoglobinuria (PNH).

    <p>False</p> Signup and view all the answers

    What symptom is characterized by high colored urine due to intravascular hemolysis during sleep?

    <p>Nocturnal Hemoglobinuria</p> Signup and view all the answers

    The mutation of the PIGA gene leads to defective _____ proteins.

    <p>GPI anchor</p> Signup and view all the answers

    Match the following clinical presentations with their descriptions:

    <p>Pancytopenia = Bone marrow may show hypercellularity or other states Aplastic Anemia = Associated with a 25/10 rule related to PNH Nocturnal Hemoglobinuria = Results in high colored urine Thrombosis = Includes hepatic, cerebral, and deep vein thrombosis</p> Signup and view all the answers

    Study Notes

    Warm Autoimmune Hemolytic Anemia (AIHA)

    • IgG is the antibody involved
    • The temperature at which the immune response occurs is 37°C
    • The anemia progresses rapidly, usually over 1-2 months
    • 50% of the cases are idiopathic
    • Secondary causes include SLE, CLL, HIV and other autoimmune diseases.

    Cold Autoimmune Hemolytic Anemia (AIHA)

    • IgM is the antibody involved
    • The temperature at which the immune response occurs is 0-4°C
    • The anemia is usually chronic and indolent
    • The idiopathic form is known as cold agglutinin disease
    • Some of the secondary causes include Waldenstrom's macroglobulinemia, lymphomas, and infections such as mononucleosis, cytomegalovirus, as well as Mycoplasma pneumonia

    Non immune Hemolysis - Fragmentation

    • The route syndrome for microangiopathic hemolytic anemia (MAHA) and thrombotic microangiopathy (TMA)
    • Observed in Hemolytic uremic syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)

    MAHA

    • MAHA is caused by endothelial injury in small vessels leading to the release of von Willebrand factor (vWF), which attracts platelets via its interaction with GP Ib-IX

    TMA

    • TMA involves small vessel disease of the kidney
    • Common causes include:
      • HUS (most important)
      • Catastrophic antiphospholipid syndrome (APS)
      • Diffuse scleroderma
      • HELLP Syndrome
      • Malignant hypertension

    Platelet plug

    • Platelets are trapped in the platelet plug, which causes thrombocytopenia

    HUS vs. TTP

    Feature HUS TTP
    Site Renal capillaries Brain (Small cerebral vessels), GIT
    Disease Renal failure Small vessel stroke/lacunar stroke
    Pentad microangiopathic hemolytic anemia, Thrombocytopenia, Fever, Neurological manifestations, Renal failure (Rare) microangiopathic hemolytic anemia, Thrombocytopenia, Fever, Neurological manifestations, Renal failure (Rare)
    Triad microangiopathic hemolytic anemia, Thrombocytopenia, Renal failure microangiopathic hemolytic anemia, Thrombocytopenia, Renal failure

    Paroxysmal Cold Hemoglobinuria (PCH)

    • Acute intravascular hemolysis
    • Polyclonal IgG antibodies bind to the P-antigen, and at 37 °C cause intravascular hemolysis
    • Coombs test is positive

    PCH Investigation

    • Donath-Landsteiner test
    • Features of warm and cold hemolysis

    PCH Associations

    • Tertiary syphilis

    PCH Treatment

    • Conservative

    Drug-Induced Hemolysis Mechanism

    • Autoantibody-mediated:
      • Drug independent:
        • Methyldopa (most common)
        • Procainamide
        • Penicillin
        • Cephalosporins
        • Lenalidomide (Cold autoimmune hemolysis)
    • Immune complex-mediated:
      • Quinidine
      • Rifampicin
      • Isoniazid
    • Adsorption:
      • High dose penicillin: Extravascular

    Coombs Test

    • Differentiates between immune and non-immune anemias
    • Direct Coombs Test (DAT): Detects antibodies on the surface of RBCs. A positive test indicates immune hemolytic anemia
    • Indirect Coombs Test: Detects antibodies in the circulation.

    Extravascular Hemolysis

    • Hemolysis happens in the spleen
    • Features:
      • Moderate splenomegaly
      • Increased mean corpuscular volume (↑ mcv)

    Approach to Hemolysis

    • Hemolysis → Hemoglobin released
    • Hemoglobin released → Reticulocytosis
    • Heme + Globin → Iron + Protoporphyrin
    • Iron → Iron stores
    • Biliverdin → Bilirubin
    • Bilirubin → Unconjugated hyperbilirubinemia → Urobilinogen and Stercobilinogen in urine and feces

    Summary of Findings

    Parameter Intravascular Hemolysis Extravascular Hemolysis
    Plasma Hb ++ -
    Urine Hb ++ -
    Urine hemosiderin ++ -
    Tissue iron - ++
    S.Ferritin ++ ++
    LDH ↓↓ ↓↓
    S.Haptoglobin ↓↓ ↑↑
    S.Bilirubin ↑ ↓↓
    Splenomegaly - ↑

    Inherited hemolytic anemia

    • Have an intracorpuscular defect, except for atypical HUS
    • Extravascular hemolysis is present, except G6PD (acute form: Intravascular hemolysis).

    Acquired causes

    • Have an extracorpuscular defect, except for PNH.
    • Intravascular hemolysis is present, except for warm and cold Ab AIHA (warm and cold: Extravascular hemolysis and Paroxysmal cold hemoglobinuria: Intravascular hemolysis).

    Warm Autoimmune Hemolytic Anemia

    • Splenic macrophages have a receptor for the Fc portion of red blood cells (RBCs), resulting in pan-agglutination, extravascular hemolysis.

    Cold Autoimmune Hemolytic Anemia

    • RBC agglutination in the peripheral circulation/extremities with IgM, recognized by Kupffer cells
    • Extravascular hemolysis is predominant, with minimal intravascular hemolysis

    Paroxysmal Nocturnal Hemoglobinuria (PNH)

    • Mutation of PIGA gene results in defective GPI anchor protein
    • Absence of complement regulatory proteins on the surface of RBCs causes complement-mediated lysis of red blood cells
    • Clinical manifestations include nocturnal hemoglobinuria, thrombosis, and pancytopenia

    PNH Clinical Presentation

    • Nocturnal Hemoglobinuria/Hemosiderinuria: Sleep → Acidotic pH → Intravascular hemolysis → High colored urine
    • Thrombosis: Frequent sites for thrombosis include hepatic venous thrombosis (resulting in Budd Chiari Syndrome), intraabdominal thrombosis, cerebral venous thrombosis, and deep vein thrombosis
    • Pancytopenia: Bone marrow: Hypercellular (most common), or normal or hypocellular
    • Aplastic Anemia: 25/10 rule
      • 25% of PNH progresses to aplastic anemia.
      • 10% of aplastic anemia is due to PNH.

    PNH investigation

    • Flow cytometry (IOC): Detects PNH III RBC's percentage. Significant: >50%
    • Urine analysis: Centrifuge urine → Supernatant → Clear→ RBC → High colored → Hb, myoglobin (Rest aside for 3-6 hrs) → Clear → myoglobin (T.: 3-6 hrs)
    • Coombs: Negative
    • Bone marrow studies
    • Hams test (Not used)

    PNH Treatment

    • Bone marrow involvement
    • Allogenic hematopoietic stem cell transplant
    • Hemoglobinuria/thrombosis
    • Anti CS antibodies (Ravulizumab > Eculizumab)
    • Meningococcal vaccine

    Note

    • ↓↓ Alkaline phosphatase level is observed in PNH and chronic myeloid leukemia (CML)
    • No scavenging due to RBC lysis also causes thrombosis.

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    Description

    This quiz covers the different types of autoimmune hemolytic anemia (AIHA), including warm and cold AIHA, as well as non-immune hemolysis like fragmentation. Learn about the antibodies involved, the conditions that cause these anemias, and key differences between warm and cold types. Test your knowledge on the underlying mechanisms and associated diseases.

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