liveonearth (liveonearth) wrote,

CPD notes review: anemias and thrombocytopenia

IDA most common

SIDEROBLASTIC anemia if high iron (all forms) but still microcytic hypocromic anemia. High RDW, anisocytosis, targeting. Definitive DX: BM: ringed sideroblasts. Look for lead toxicity.

ACD = 2nd most common form, may/may not occur w/ IDA, mechanism is short RBC life, impaired iron metabolism and deficient erythropoeisis, normochromic normocytic w/ normal RDW initially but progresses to micro-hypo.

Hypoproliferative dt low EPO or damaged marrow

APLASTIC anemia = bone marrow mass decrease, pancytopenia or pure red aplasia, sometimes idiopathic in young adults, oft dt chem, age, FANCONI's (genetic), pure red aplasia mbdt infx, thymoma, immune, fertilizer, B2 deficiency, CLL. S/Sx: pallow, thrombocytopenia, agranulocytosis-->infx. Labs: low cells high iron acellular marrow.

MYELOPHTHISIC anemia = marrow replaced by tumor, granuloma, lipid storage, fibrosis, S/Sx: those of underlying dz and/or splenomegaly. Labs: aniso & poikilocytosis, nucleated RBC's, immature myeloid cells in PB, bone marrow is definitive Dx.

MACROCYTIC/MEGALOBLASTIC anemia = dt defective DNA syn w/ normal RNA syn. ETIO: B12/folate deficient, cytotoxic drugs. S/Sx: neurological, stocking glove paresthesia, dementia, neuropathy, glossitis, diarrhea, muscle wasting. Labs: MCV > 95-100, aniso, poik, high RDW, Howell-Jolly bodies, hypersegs early, neutropenia later. Increase in retics after B12 injection when that's what pt needed.

THROMBOCYTOPENIA S/Sx: easy ecchymosis, bruising/bleeding esp of mucosa, gingiva, ocular fundi, petechiae

HEMOLYTIC anemias --- intrinsic RBC probs: membrane, metabolism or Hgb synthesis, extrinsic probs: hypersplenism, autoimmune, mechanical, infx. Congenital membrane disorders: porphyria, elliptocytosis, spherocytosis. Acquired membrane prob: hypophosphatemia, paroxysmal noscturnal hemoglobinuria, stomatocsytosis. Metabolism prob: Embden-Meyerhof and G6PD. Hgb syn probs: HbC, HbS-C, HbE dz, Sickle, thalassemia. LAbs: elevated bilirubin and LDH, normal ALT, CHECK URINE FOR HEMOSIDERIN, HGB, and SERUM HAPTOGLOBIN if decreased you have intravascualr hemolysis. (Haptoglobin binds to free HGB)

COOMB'S test = checks for autoimmune reason for hemolysis

AUTOIMMUNE hymolytic anemia: warm or cold, Warm more common, more in women, ideopathic or SLE, lymphoma, CLL (both these mb lymphoprolif), drugs. S/Sx: anemia, mild splenomegaly, if severe fever, chest pain, syncope, heart failure. Cold Ab dz, see infx (mycoplasma) or lymphoproliferative state, hemolysis mostly in liver w/ mild anemia, acrocyanosis, Raynaud's. Dx: Coomb's test.

HEREDITARY SPHERO- & ELIPTO- CYTOSIS = congenital lack of right structure in RBC's---they don't bend and pop too easily. Aka: chronic familiar icturs, congenital hemolytic jaundice). Both kinds are autosomal dominant, ellipto is more rare. S/Sx: gallstones, jaundice, anemia, mb hepatomegaly. Dx: funny shaped cells, normal or low MCV, elevated MCHC, retics or leuks mb elevated, increased LDH, DECREASED HAPTOGLOBIN. Tx is splenectomy.

STOMATOCYTOSIS = rare, congenital or acquired RBC membrane disorder, congenital presents early, acquired dt big alc consumption, Sx: anemic w/ RBCs w/ straight or rectangular area of central pallor

HYPOPHOSHPATEMIA => low ATP --> RBC's are rigid, small, spheroid

G6PD DEFICIENCY = dt genes (x-linked, more in Afro males some in medits) or drugs, hemolysis when cells exposed to oxidant such as drug, fava beans, vit K derivatives, salicylates, sulfonamides. Triggers: stress, fever, acute infx, diabetes. S/sx: anemia, jaundice, renal failure. Labs: HEINZ BODIES, retics up, bit cells. Dx: G6PD assay.

SICKLE CELL ANEMIA = S/Sx: bone pain crises, renal damage, punched out leg ulcers, splenic infarcts, aplastic crises, anemia usu severe, developmental delays, avascular necrosis of femoral head, severe abdom pain w/ vomiting, neuro disturb, splenomegaly. Death dt infx, pulmonary emboli, vessel occulsion. If only trait, S/sx: myalgias, hematuria during hypoxia. LABS: low RBC, Hct, Hgb, normal or high WBC's, normocytic anemia, elevated bilirubin, nucleated RBC's, sickle cells, high plasma HGB. Dx: Hgb electrophoresis. Pt build: short trunk, long limbs.

HGB C DZ = another in Africans, variable, S/Sx: bone pain, splenomegaly, mild jaundice, anemia. LABS: normochromic w/ target cells, SPHEROCYTES

LEUKOPENIA = < 4,000 WBCs/mm3. Levels over 1200 in blacks or 1500 in whites mb ASx. Under 500 severe risk, under 200 rapidly fatal infx. Profound neutropenia-->cellulitis, liver abscess, furunculosis, pneumonia, septicemia. If drug induced may have rash, LAD.

LYMPHOCYTOPENIA = < 1000 in adults, 3000 in children under 2. 75% T cells, 25% B cells. Acquired: HIV, hep, flu, TB, typhoid, sepsis. S/sx: from primary dz.

THROMBOCYTOPENIA = low platelets. Normal is 140,000-440,000, varies by menstrual cycle, decreases in preg near term, increases w/ inflam. Platelet lifespan: 7-10 days. Easy minor bleeding under 50,000, at 5,000 mb more serious, some with less than 10,000 mb Asx. Dx: CBC, coag studies, PB smear. Suspect platelet dysfunction if CBC, INR and PTT are normal. ETIO if acquired: aspirin, NSAIDS, very common, effect lasts 5-7 days. MOA: prevent production of Thromboxane A2.

INR/PT = test for coagulation, extrinsic and common pathways (VII, X, V), prothrombin, fibrinogen, INR is standardized to help with Warfarin dosing. In vivo coagulation is mostly extrinsic.

PTT = another coag test, intrinsic and common pathways (XII, XI, IX, VIII, X, V), prolonged by heparin, warfarin/coumadin, also by auto-ab's to factor VIII (hemophilia). Factors must be at least 70% deficient to see prlonged PT/PTT.

HEREDITARY INTRINSIC PLATELET DISORDERS: three main types: poor activation, Glanzmann's Thromboasthenia (rare, autosomal recessive, severe mucosal bleeds dt no aggregation), Bernard-Soulier syndrome (also rare, auto-recess, LARGE platelets, impaired adhesion, severe bleeds) Dx: platelet aggregation tests

ITP = IDIOPATHIC THROMBOCYTOPENIC PURPURA = autoimmune w/ platelet destruction, mb triggered by viral infx in kids, chronic in adults, S/Sx: peteichiae, mucosal bleeding, splenomegaly for kids. Dx: unexplained thrombocytopenia, low PLT otherwise normal CBC, bone marrow exam: normal or increased megakaryocytes

THROMBOCYTOPENIA dt SPLENIC SEQUESTRATION, PLT under 30,000, in anything with sequestration including cirrhosis, myelofibrosis, myeloid metaplasia

THROMBOTIC THROMBOCYTOPENI PUPURA = microangiopathic TT = platelets destroyed by fibrin in small vessels-->thrombi in multi organs. Grouped with HUS. ETIO: ADAMTS13 enzyme deficient in plastma, hemorrhagic colitis from E. Coli 0157 or some shigella, preg, drugs (quinine, cyclosporine, mitomycin C), idiopathic. S/Sx: fever, organ ischemia sx. Dx: suspect w/ anemia, thrombocytopenia, and sx. LABS: US, PB smear, Retic ct, serum LDH, renal fx, serum bilirubin, Coomb's test. DX SUGGESTED BY: anemia, RBC fragments, hemolysis (falling HGB, polychomasia, elevated retics, negative Coombs)

HEMOLITIC UREMIC SYNDROME = TTP in kids dt EHEC or shigella, usu spontaneously remits, untx mb fatal, usu only one incidence

VON WILLEBRAND'S DZ = PROBLEM WITH VW FACTOR which promotes platelet adhesion by binding platelets to endothelium of vascular sys. Hereditary, impaired syn of VWF->long bleed time, mild-severe factor 8 deficiency. Autosomal dominant inheritance. S/Sx: usu mild-mod, skin & mucosal bleeds, easy bruise, menorrhagia, prolonged bleeds after surgery. Dx: suspect in those w/ fam hx or personal hx of bleeding dz, coag tests mb normal (normal INR/PT, slighly prolonged PTT), normal PLT count, DEFINITIVE: test for plasma VWF antigen, VWF fx, and Factor 8. VWF antigen mb 40% below normal in HEALTHY peerson with TYPE O BLOOD. Beware of ASA (aspirin)

GENERAL S/SX OF BLEED DISORDER: unexplained epistaxis, menorrhagia, prlonged bleeds after minor trauma, easy bruisng, petechiae, unexplained gingival bleeds, hx of bleeds, nsaids intake, menorrhagia, hematuria, GI bleeds, hemoptysis

BLEEDING TIME TEST = controversial, used more by naturopaths than conventional docs, multifactorial but useful for helping pt w/ longterm maintenance of clotting level, 6-8 mins optimal, 2-10 normal.
Tags: anemia, blood, diagnosis, microbes, nd2, spleen

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