Hemonc
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  • Anemia (Hb--): fatigue because Hb--.
    • Hypoproliferative anemia (underproduction): low retic anemia.
      • Megaloblastic anemia (malnutrition): lack Folate, B12 (neuro).
        • Folate deficiency: macrocytic anemia, Hx of EtOH, diet lacking vegees, dx w serum homocysteine↑, folate↓.
        • Vitamin B12 deficiency (cobalamine): macrocytic anemia + neuro, Hx of being vegetarian, malabsorptive dz (Crohn, bypass), dx w homocysteine↑ methylmalonic acid↑, B12↓.
      • Iron deficiency (pica): pica, microcytic anemia with low ferritin.
      • AoCD: infection, fatigue, low blood Fe, high stored Fe (ferritin), don't want Fe (TIBC↓).
      • Aplastic crisis (B19): Parvo B19 (suppress erythropoiesis) on top of a hemolytic anemia (sickle, thalassemia, spherocytosis, G6PD) = no RBC.
    • Hemolytic anemia: High retic anemia, frontal bossing, RBCs lysis (Hb↑, LDH↑, unconj Hb↑, liver conj→brown urine).
      • Hereditary spherocytosis (spectrin): weak membrane -> spheres -> lyse.
      • G6PD deficiency (bite): fava beans and drugs trigger hemolytic anemia with bite cells.
      • Warm agglutinins (IgG): exertional dyspnea due to IgG anti-RBC.
      • Cold Agglutinins (IgM): Cold triggers blue fingers, ears because of autoimmune IgM.
      • MAHA (schistocyte): shear (schistocyte) and tear (LDH++, bili++) of RBCs due to DIC, TTP/HUS.
      • Hemoglobinopathy
        • Quantitative hemoglobinopathy (thalassemias): alpha, beta thalassemias.
          • Alpha thalassemia (newborn): anemic newborn with hemolysis (jaundice, liver++, spleen++).
          • Beta thalassemia (infant): anemic infant with hemolysis (jaundice, liver++, spleen++).
        • Structural hemoglobinopathy (glu6): HbS, HbC.
          • Sickle cell anemia (vaso-occlusive): African Am with a bunch of vaso-occulsive problems (pain, thrombosis, stroke, priapism, spleen infarct/asplenia).
          • Hemoglobin C Disease (xtal): African Am with hemolytic anemia and xtals and target cells.
  • Thrombocytopenia (plt--): underproduction (marrow), consumption (DIC, TTP), splenomegaly (sequest), heparin-induced, ITP.
    • DIC (shock): bleeding (petechiae, wound), organ failure, shock.
    • TTP/HUS: bad plasma (VWF↑, ADAM13↓) causes clot and MAHA, so tx with plex (not plt txf).
    • ITP: female, petechiae, bleeding, low plt, dx of exclusion, tx steroid..
    • Purpura Fulminans (DIC): DIC (petechiae) -> purpura (necrosis + bleed).
  • Bleeding
    • VWD (desmopressin): the hemophilia that can present in females, affects both plt and factor 8, tx with DDAVP.
    • Hemophilia A (bleed): factor 8 bleed.
    • Hemophilia B (bleed): factor 9 bleed.
  • Thrombophilia (clot): clot++ (PT 202101), anticlot-- (prot C,S, Antithrombin)--, resist anticlot (factor 5 leiden resist prot C), antibodies (lupus, cardiolipin).
  • Immunodeficiencies: frequent infections
    • B cell
      • Bruton's agammaglobulinemia: infections after 6 mo (maternal Ig weans), no tonsils or adenoids, X-link FHx, antibodies↓ for all Ig classes.
      • Hyper IgM syndrome: infections, IgM↑, other Ig classes↓, due to defect CD40L on helper T (IgM B can't class switch).
      • Selective IgA deficiency: URTIs, GI infections, allergies, IgA↓.
      • CVID: infections, antibodies↓ (all Igs, esp IgG), plasma cells↓, normal B count, bcz immature B can't mature.
    • T cell
      • DiGeorge syndrome: no thymus (infections, T cell↓), no parathyroids (tetany, Ca↓, PTH↓), cardio (tet of Fallot, cyanosis), facial (eyes wide apart, cleft palate), due to 22q11 del.
      • IL 12 receptor deficiency: severe mycobacteria & salmonella infections.
      • Hyper IgE syndrome: FATED: Facies (course), Abscesses (cold, staph), Teeth (retained primary), E (IgE), Derm (eczema), due to lack of IFN-γ (no chemotaxis).
      • Chronic mucocutaneous candidiasis: candidiasis of skin & mucous membranes due to T cell dysfunction.
    • B & T cell
      • Adenosine deaminase deficiency: imbalance of too much ATP, dATP = messes up DNA synthesis = SCID.
      • Ataxia telangiectasia: ataxia (gait, coord), telangiectasia (eye, skin), infections, malignancy, AR FHx, IgG↓, IgA↓, AFP↑.
      • Wiscott-Aldrich syndrome: plt↓ (petechiae, bruising), infections (WBC↓), eczema, X-link FHx (boys).
    • Phagocytic dysfunction (infection): diseases that affect phagocytes: LAD (PMN++), CGD (staph), MPO (fungi), CHS (pyogenic).
      • LAD: infections w/o pus, WBC↑, PMN↑, delayed cord separation, bcz lack integrin, WBCs stuck in circulation.
      • CGD: phagocytes eat but can't kill (no NADPH, granuloma) leading to infections (pneumonia, skin) by catalase+ bugs (staph)..
      • MPO deficiency (fungal): can't kill fungi (candida) bcz no MPO (stain PMNs).
      • Chediak-Higashi syndrome: infections (pyogenic, staph, severe), albinism (partial), due to microtubule prob (no phagolysosome formation), dx w granules in PMNs, giant melanosomes in skin/hair.
  • Myeloproliferative disorders (basophilia): Stell cells gone wild (basophil++).
    • Polycythemia vera (Hct++): headache, itch, pins-and-needles, and Hct++.
    • Essential thrombocythemia (plt++): plt++ but not from CML (phil chr) or reactive (inflame,Fe def).
    • Myelofibrosis (teardrop): fatigue, splenomegaly and teardrop cells.
    • CML: tired pt with basophilia and Bcr-Abl = t9; 22 = phil chr, tx w imatinib/gleevec (inh bcr-abl).
  • Myelodysplastic (pancytopenia): pancytopenia: RBC (anemia), PMN (infections), plt (bruise) with dysplastic marrow biopsy.
  • Leukemia (WBC): pt comes in with B symptoms (fever, sweat, wt loss), high white count, and abnormal blood WBCs.
    • ALL/LBL (kids): B symptoms (fever, sweat, wt loss), marrow problem (anemia/tired, plt--/bleed, PMN--/infection) in kids due to immature blasts (lymphoid, vs AML).
    • CLL/SLL (smudge): lots of mature lymphocytes that suck (node++, IgG--/infection, AIHA) and that smudge cell.
    • AML (Auer): myeloid won't differentiate (anemia/tired, PMN--/infection, plt--/bleed), and that auer rod nails it..
    • CML: tired pt with basophilia and Bcr-Abl = t9; 22 = phil chr, tx w imatinib/gleevec (inh bcr-abl).
  • Lymphoma (node++): cancer in lymph nodes (painless node++) that is either low grade (SLL, follicular), or high grade/B symptoms (burkitt, LBL, diffuse large, hodgkin).
    • Hodgkin's lymphoma (Reed-Sternberg): HIV, EBV, cervical/mediastinal node++, Reed-Sternberg, cured by ABVD+rad..
    • Non-Hodgkin's lymphoma (mesenteric): B,T cell cancer in nodes: can be low grade/insidious (SLL, follicular), or high grade/aggressive (burkitt, LBL, diffuse large).
      • Follicular lymphoma: low grade/asymptomatic, painless lymph node, monoclonal B (BCL-2), biopsy has centrocytes (small cleaved).
      • DLBCL (R-CHOP): B symptoms (fever, sweat, wt loss), lymph node↑ (aggressive), biopsy shows large blast-like cells, tx w R-CHOP.
      • Burkitt's lymphoma (starry-sky): facial tumor (African), GI/pelvic (sporadic), node++ (aggressive), starry sky node biopsy, c-MYC.
      • ALL/LBL (kids): B symptoms (fever, sweat, wt loss), marrow problem (anemia/tired, plt--/bleed, PMN--/infection) in kids due to immature blasts (lymphoid, vs AML).
      • CLL/SLL (smudge): lots of mature lymphocytes that suck (node++, IgG--/infection, AIHA) and that smudge cell.
  • Plasma cell dyscrasia (M-protein): plasma cell cancer.
    • Multiple myeloma (CRAB): CRAB (Ca++, Renal, Anemia, Bone) with M protein and marrow plasma cells++.
    • MGUS (3/10): not quite MM (no CRAB, M protein < 3g/dL, marrow plasma cells < 10%), but getting there (1%/year).
    • Amyloidosis: M protein LC (immunofix) forms amyloids (congo red/apple green) that mess up organs (renal, liver, cardio, neuro, tongue, MSK).
    • Waldenstrom's macroglobinemia (lymphoplasma): anemia (tired), hyperviscosity (headache/dizzy) and retinal problems (sausage, clot) due to IgM M proteins (AI) made by lymphoplasma cells (>10% marrow).
  • High grade: more B symptoms, rapid lymph node growth, curable
    • ALL/LBL (kids): B symptoms (fever, sweat, wt loss), marrow problem (anemia/tired, plt--/bleed, PMN--/infection) in kids due to immature blasts (lymphoid, vs AML).
    • Burkitt's lymphoma (starry-sky): facial tumor (African), GI/pelvic (sporadic), node++ (aggressive), starry sky node biopsy, c-MYC.
    • DLBCL (R-CHOP): B symptoms (fever, sweat, wt loss), lymph node↑ (aggressive), biopsy shows large blast-like cells, tx w R-CHOP.
    • Hodgkin's lymphoma (Reed-Sternberg): HIV, EBV, cervical/mediastinal node++, Reed-Sternberg, cured by ABVD+rad..
  • Low grade: less B symptoms, slow lymph node growth, incurable
    • CLL/SLL (smudge): lots of mature lymphocytes that suck (node++, IgG--/infection, AIHA) and that smudge cell.
    • Follicular lymphoma: low grade/asymptomatic, painless lymph node, monoclonal B (BCL-2), biopsy has centrocytes (small cleaved).