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Hemolytic anemia (:white_flower:Approach to anemia in children (:four_leaf…

- - - - Ethnic factors (eg High incidence of thalassemia trait in ppl)
      - Fx Hx of anemia, jaundice or gallstones
      - Anemia assoc. with Reticulocytosis
      - Intermittent or Persistent Indirect Hyperbilirubinemia
      - Splenomegaly
      - Hemoglobinuria
      - presence of multiple gallstones
      - Development of anemia or hemoglobinuria after exposure to Certain Drugs
    - - :arrow_down: red cell survival and evidence of
        accelerated Hb catabolism :astonished:
        
        :astonished: Intravascular Hb catabolism signs
        
        Low or Absent plasma haptoglobin
        
        Haptoglobin
        (for Acute Intravascular hemolysis)
        
        In Acute Intravascular hemolysis,
        hap & Hb are destroyed ➯ hap= 0 or < 20
        
        Normally it’s protein binding to free Hb
        
        Hemoglobinemia
        
        Hemoglobinuria
        
        Hemosiderinuria
        
        :astonished: Extravascular Hb catabolism signs
        
        :arrow_up: unconjugated bilirubin
        
        :arrow_up: fecal & urinary urobilinogen
        
        :arrow_up: rate of Carbon monoxide production
      - Reticulocytosis (freq up to 10 - 20%)
        evidence of :arrow_up: erythropoiesis
  - - - Acute
        (< 7 days)
        
        Acute blood loss
        
        Acute hemolysis
        
        Intravascular
        ( :black_small_square: coke color urine)
        
        G6PD def
        
        Mismatch
        
        Extravascular
        (spleen & RE system — Macrophage destroy RBC)
        
        HbH with crisis
        
        HS with crisis
        
        AIHA
      - Chronic
        (> 2 months)
      - Subacute
        (7 days - 2 months)
    - - :arrow_down: production
        
        Impaired production: Iron def , Folate def
        
        BM failure: Aplastic anemia, BM replacement—eg CA cells ไปแออัดเบียดที่ BM เลยไม่มีที่สร้าง
      - Hemolysis
        
        Def: premature destruction of erythrocytes, reduction in normal RBCs survival of 120 days
        
        :chicken:Causes
        
        Corpuscular abnormalities
        (within the red cell corpuscle)
        
        Membranes abnormalities
        
        Metabolic/enzymes abnormalities
        
        Hemoglobinopathies
        
        ExtraCorpuscular abnormalities
        (immune or nonimmune mech)
        
        Non immune
        
        Immune
      - Blood loss
    - - Microcytic (MCV < 80)
        
        Iron def #
        
        Thalassemia
      - Normocytic
        
        Anemia of chronic ds
        
        Aplastic anemia
        
        Acute blood loss
        
        Other hemolytic anemia
      - Macrocytic (very rare)
        
        Megaloblastic anemia (B12 def, Folate def)
- - - - GI bleeding (ถ่ายดำ เลือดปนหรือไม่)
      - Menses (age ≥ 9 y/o)
  - - - Eye: cataract
      - Face: frontal bossing
      - Mouth: glossitis/ลิ้นเลี่ยน:comet:, angular stomatitis
- - - - Allo immune hemolytic anemia
        (Ab คนอื่นมาทำลาย)
        
        1.Hemolytic ds of newborn (HDN)
        
        :gear: Mech: Maternal IgG Ab directed against Paternal origin Antigen present on fetal RBCs ➯ IgG Ab cross the placenta to coat fetal Ag ➯ :arrow_down: RBC survival ➯ anemia
        
        :octopus:Classifications
        
        ABO incompatibility
        
        Mom group O (got Anti-A, Anti-B) AND Baby group A or B
        ➯ mom Anti-A, Anti-B vines to fetal Antigen on its RBCs
        
        Minor blood group incompatibility— Jk, K, Fy, S etc
        
        Rh - anti-D
        
        2.Transfusion reaction (mismatch)
      - Auto immune hemolytic anemia
        (Ab ตัวเองมาทำลาย)
        
        :balloon:Types
        divided base on Ability of autoAb to fix complement and site of RBCs destruction
        
        :sunny: Warm type
        
        Usu. IgG Ab
        
        Fix complement only to Level C3 if at all (not complement fixing)
        
        IgG bindings occurs at All temp
        
        Fc receptors/C3b regconized by macrophages
        
        primarily Extravascular hemolysis
        
        70% assoc with other illnesses
        
        Responsive to Steroids/Splenectomy
        
        Optimal temp 37°C
        
        Non-agglutination
        
        :snowflake: Cold type
        
        M/C IgM Ab
        
        Abs bind best at temp ≤ 30°C
        
        Fix entire complement cascade (fix complements)
        
        Primarily Intravascular hemolysis in vivo
        (membrane attack complex formation ➯ Intravascular RBCs lysis)
        
        Typically only complement found on cells
        
        90% assoc. with other illnesses
        
        Poorly response to Steroids, splenectomy
        responsive to Plasmapheresis
        
        Optimal temp 0 - 4 °C
        
        Strong agglutination
        
        :wind_chime:Def
        
        Due to formation of AutoAb that attack pt’s own RBCs
        
        Often assoc. with either Lymphoproliferative ds or Collagen vascular ds
        
        :cake: Causes
        
        Primary or Idiopathic AIHA
        
        Secondary AIHA — AIHA associated with..
        
        rhuematic disorders esp SLE
        
        infections
        
        non-lymphoid neoplasm (eg ovarian cancer)
        
        chronic inflammatory ds (eg ulcerative colitis)
        
        drugs (eg alpha-methyldopa)
        
        lymphoproliferative disorders
        
        :evergreen_tree: Mx
        
        Steroid is the mainstay Rx
        
        Doses
        
        Prednisolone 1 - 1.5 mg/kg/day PO x 4-6 wk
        
        response usu. in 1st wk then taper
        
        overall response rate 75%
        
        Pulse methyprednisolone 1g/kg/day x 3 days
        for severe hemolysis
  - - - :lower_left_crayon: Thalassemia
        
        :wind_chime: Def
        
        Most
        
        α thal are due to deletion of α-globin genes (rather than point mutation)(chromosome 16)
        
        β thal are due to point mutations in β-globin genes (chromosome 11)
        
        Normal type of Hb levels
        
        Hb A 97%
        
        Hb A2 < 2.5%
        
        Hb F < 1%
        
        α-thalassemia severity:
        
        Silent carrier α-thalassemia
        
        α-thalassemia trait
        
        Hb Constant spring
        
        HbH ds
        
        Hydrops fetalis
        
        β-thalassemia
        
        β0 thalassemia (no detectable β-chain synthesis due to absent β-chain messenger RNA (mRNA))
        
        β+ thalassemia ( :arrow_down: β-chain synthesis due to reduced or nonfunctional β-chain mRNA)
        
        βE thalassemia : HbE (lysine replaced by Glutamic acid at codon 26)
    - - :lower_left_crayon: G6PD deficiency
        
        :wind_chime: Def
        
        XR inheritance
        
        Glucose-6-phosphate dehydrogenase — first enzyme in PPP of glucose metabolism
        
        Deficiency of this G6PD enz ➯ :arrow_down:energy of RBCs ➯ Hemolysis
        
        During stress/infection/oxidation ➯ :arrow_up:Hydrogen peroxidase ➯ not enough GSH (that body usu create to compensate) ➯ destroy RBCs of pt
        
        :frame_with_picture:Clinical
        
        Neonatal Jx
        
        Acute hemolytic anemia from drugs — Primaquine, Sulfamethoxazole, Dapsone
        :warning: Beaware of Acute renal failure
        
        Exaggerated Jx in Acute viral hepatitis
        
        Female: X-inactivation (X 1 ข้างไม่ทำงาน ดังนั้น X อีกข้างที่เป็นพาหะเลยทำงาน)
        
        :gear: Pathogenesis: RBC’s G6PD activity fall rapidly & prematurely RBCs age ➯ :arrow_down: glucose metabolism ➯ diminished NADPH/NADP & GSH/GSSG ratios ➯ Impaired elimation of oxidants (H2O2) ➯ Oxidation of Hb and of sulfhydryl groups in the membrane ➯ impaired Red cell integrity esp on exposure to Oxidant drugs and chemicals
        
        Oxidative stress ➯ Hb precipitation
        
        Drug-induced
        
        Primaquine (antimalarial)
        
        Sulfa drugs including co-trimoxazole
        
        Methylene blue
        
        Naphthalene
        
        Note: during acute hemolysis, many Premature RBC comes out ➯ So ช่วงแรกของ Intravascular hemolysis — level of G6PD will be normal ➯ *must wait for another 3 months then เจาะเลือดดูใหม่
        
        Blood film
        
        Basket cell, bite cells,
        
        Heinz bodies (in special stain)
    - - :lower_left_crayon:Hereditary spherocytosis
        (HS)
        
        Blood Pics
        
        No central pallor (think of polychromasia and microspherocyte)
        
        Spherocyte size as RBCs
        
        :wind_chime:Def
        
        Normally Spectrin maintain RBC shape & compose of two subunits —alpha- and beta-spectrin
        
        A variety of mutations in alpha- and beta- spectrin assoc. with abnormally shaped RBCs
        
        AD inheritance (75% of cases)
        
        Extravascular ➯ RBC destroy at spleen
        
        :gear:Pathogenesis: :arrow_down:RBCs deformability ➯ sequestration of RBCs in spleen ➯ :arrow_down: membrane surface area ➯ :arrow_up: influx and efflux of Sodium
        ➯ cell dehydration ➯ Rapid ATP utilization & :arrow_up:glycolysis
        ➯ Premature RBCs destruction
        
        :frame_with_picture:Clinical features
        
        Anemia + Jaundice
        
        Splenomegaly
        
        most pt presents before puberty
        
        :male-scientist::skin-tone-2:Laboratory findings
        
        MCV usu :arrow_down:; :arrow_up:MCHC & RDW
        
        Reticulocytosis (3 -15%)
        
        Blood film: Microspherocytes, Polychromasia
        
        Coombs test :heavy_minus_sign:
        
        :arrow_up: red cell osmotic fragility — for Dx
        (spherocytes lyse in higher conc of saline than normal RBCs, แตกเร็วกว่าคนปกติ) :grapes:
        
        Notes: Thalassemia OF test จะแตกช้ากว่า normal, Hereditary spherocytosis จะแตกเร็วกว่า normal RBCs
        
        :evergreen_tree: Mx
        
        Blood transfusion if Hb < 7
        
        Folic a supplementation
        (*no need for iron)
        
        Splenectomy in Severe cases
      - Hereditary elliptocytosis
      - Hereditary stomatocytosis
      - Hereditary pyropoikilocytosis