Docstoc

APPROACH TO PATIENT WITH HEMOLYTIC ANEMIA

Document Sample
APPROACH TO PATIENT WITH HEMOLYTIC ANEMIA Powered By Docstoc
					APPROACH TO A PATIENT
   WITH HEMOLYTIC
       ANEMIA

            By
     Dr. Ramlah Hasan
         H.O MU-II
                Definition
Hemolytic are a group of disorders characterised
  by shortened survival of RBCs in circulation .
             An introduction
                               Consequences of hemolysis
  Shortening of red cells survival does not always cause
     anemia as there is a compensatory increase in red cell
 production by the bone marrow.If the red cell loss can be
      contained within the marrow’s capacity for increased
    output,then a hemolytic state can exist without anemia
  (compensated hemolytic disease). The bone marrow can
       increase its output by 6 to 8 times by increasing the
proportion of cells committed to erythropoiesis (erythroid
       hyperplasia) and by expanding the volume of active
    marrow. In addition, immature cells (reticulocytes) are
                                        released prematurly.
        Sites of hemolysis

                Extra vascular hemolysis
   In most hemolytic conditions red cell
     destruction is extra vascular. The red
Cells are removed from the circulation by
  macrophages in the reticuloendothelial
           system, particularly the spleen.
Sites of Hemolysis (contd.)
                      Intra vascular Hemolysis
    When red cells are rapidly destroyed within
  the circulation Hb is liberated. This is initially
     bound to plasma haptoglobin but these soon
     become saturated. Excess free plasma Hb is
 filtered by the glomerulus and enters the urine,
  although small amounts are reabsorbed by the
    renal tubules. In the renal tubular cells Hb is
      broken down and becomes deposited in the
                            cells as haemosiderin.
Classification of Hemolytic
            Anemias
                          Intra corpuscular
                                             Hereditary
                                  Membrane Defects
                             Hereditary Spherocytosis
                             Hereditary Elliptocytosis
                                 Enzyme Deficiency

                                   G6PD Deficiency
                          Pyruvate Kinase Deficiency
                               Haemoglobinopathies
                                       Sickle Cells Anemia
                                    Unstable Haemoglobins
                                                 Acquired
             Paroxysmal Nocturnal Haemoglobinuria
Classification of Hemolytic
          Anemias
                                  Extra corpuscular
               Autoimmune Hemolytic Anemias
                       Associated with warm antibodies
                         Associated with cold antibodies

                 Isoimmune Hemolytic Anemias
               Drug induced Hemolytic Anemias
                                    Infections
                                 Plasmodiam, Clostridium
         Microangiopathic Hemolytic Anemias
                              Hypersplenism
Clinical Approach
                    History
               Examination

               Investigation

                  Treatment
                History
                                                    Age
               Hemolytic anemia can occur at any age.
   Hereditary disorders usually are evident early in life.
 AIHA is more likely to occur in middle-aged and older
                                            individuals.
                                                   Sex

 Most cases of hemolytic anemia are not specific to any
                                                 gender.
Autoimmune hemolytic anemia (AIHA) is slightly more
               likely to occur in females than in males.
  G-6-PD deficiency is an X-linked recessive disorder.
    Males usually are affected, and females are carriers
             History (contd.)
                                           Symptoms
    Pallor, Fatigue, Worsening of preexisting, angina,
                         Palpitation, Postural dizziness
                      Mottled or Numb fingers or toes

                                    Pigment gall stone

                   Reddish Brown first morning urine

        Symptoms due to splenomegaly like dragging

                                  sensation in abdomen
              Acute painful episode include the bones

    (especially the back and long bones) and the chest.
       History (contd.)
                           Family History
   Positive Medical History for Associated
                                  Diseases
                             Drug Hsitory

                                  Analgesics
                               Anti-malarials

                               Anti-bacterials
       Examination
                                   Pallor
                                Jaundice

                            Tachycardia

                          Flow murmurs

      Signs of congestive cardiac failure

   Poorly healing ulcers over lower tibia
                          Splenomegaly

                           Cholelithiasis
 Investigations
                                    Blood Complete Picture
                                                              Hb  or N
                                                                  TLC

                                                                  PLT

                                                 Red Cell Indices
                                                                       MCV
                                                                      MCHC
                                           Reticulocytosis
                                              

                                Bone Marrow Examination
                                         Erythroid Hyperplasia
                                                     Serum Levels
                                                      Bilirubin
                                                           

The level of unconjugated bilirubin never exceeds 4 to 5 mg/dl.
                                                Haptoglobin

                                       Plasma Haemoglobin
                                      Lactate Dehydrogenase

                            Positive Urinary Haemosiderin
         Peripheral Blood Film
                 Blood Film

 Spherocytes No spherocytes       Fragmentation

DCT+    DCT-             Hereditary
                       enzymopathies
Autoimmune      Hereditary       Microangiopathic,
 hemolysis     spherocytosis         Traumatic

       Malaria, Clostridum
Hereditary Spherocytosis
Sickle Cell Anemia
Other Investigations
              Osmotic fragility test
          Specific enzymatic assays
                Hb electrophoresis
           Evaluation of Hemolysis
                        Extra vascular   Intra vascular
Plasma Or Serum
Bilirubin                Unconjugated   Unconjugated
Haptoglobin             , Absent        Absent
Plasma Hb               N-              
Lactate Dehydrogenase   ) variable)       (variable)
Urine
Bilirubin               0                0
Hemosiderin             0                +
Haemoglobin             0                + in severe cases
                        Treatment
    More than 200 types of hemolytic anemia exist, and each type
    
requires specific treatment. Therefore, only the aspects of medical
   care relevant to most cases of hemolytic anemia are discussed.
                                             Transfusion therapy
  Avoid transfusions unless absolutely necessary, but they may be essential for
         patients with angina or severely compromised cardiopulmonary status.
                       Administer packed RBC slowly to avoid cardiac stress.

    In AIHA, type matching and crossmatching may be difficult. Use the least
 incompatible blood if transfusions are indicated. The risk of acute hemolysis of
    transfused blood is high, but the degree is dependent on the rate of infusion.
Slowly transfuse by administering half units of packed red cells to prevent rapid
                                                  destruction of transfused blood.
                                                Discontinuing medications
   Discontinue penicillin and other agents that can cause immune hemolysis and
                                           oxidant medication such as sulfa drugs
               Treatment (contd.)
  Administer folic acid because active hemolysis may consume folate
                                              and cause megaloblastosis.
                               Corticosteroids are indicated in AIHA.

 IV immunoglobulin G (IgG) has been used for patients with AIHA,
    but only a few patients have responded, and the response has been
                                                                transient.
                                                           Iron therapy
  This is indicated for patients with severe intravascular hemolysis in
     which persistent hemoglobinuria has caused substantial iron loss.
        Before iron is administered, document the iron deficiency by
     serum iron studies and, possibly, by assessing iron stores in bone
                                                        marrow aspirates.
       Because iron stores increase in hemolysis, iron administration
       generally is contraindicated in hemolytic disorders, particularly
                          those that require chronic transfusion support.
             Treatment (contd.)
                                       Surgical Care:
                                           

   Splenectomy may be the first choice of treatment in
     some types of hemolytic anemia such as hereditary
                                        spherocytosis.
       In other cases, it is recommended when other measures,
                                   such as in AIHA, have failed.
        Splenectomy usually is not recommended in hemolytic
            disorders such as cold agglutinin hemolytic anemia.
     Immunize against infections with encapsulated organisms,
             such as Haemophilus influenzae and Streptococcus
     pneumoniae, as far in advance of the procedure as possible.
      Points to Remember
   Peripheral smear is the most important
                   initial investigation and
      Coombs test with anti-IgG and anti-
      complement in diagnosing hemolytic
                                    anemia.

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:256
posted:4/6/2011
language:English
pages:22