EM E2: Heme-Onc

What are the MC causes of anemia in the US?

iron deficiency, thalassemia, anemia of chronic disease

What are sx of anemia?

tachycardia, cold extremities, pale conjunctiva

Would acute or chronic anemia show sx first?

Acute

What sx of chronic anemia?

weakness, fatigue, lethargy, dyspnea, palpitations, orthostatic sx

What are sx of acute anemia?

hypotension, thirst, dec urine output

What labs values indicate anemia?

dec RBC, hemoglobin & hematocrit

What work up is needed to help determine the cause of anemia?

MCV, RDW, GI stool for occult blood

What is the tx for anemia?

based on sx and cause

ED would see acute blood loss → transfusion of PRBC’s

Which anemias are microcytic?

IDA, chronic infxn, Thalassemias, Hemoglobinopathies, Sideroblastic anemia

What anemias are normocytic?

chronic disease, early IDA, hemoglobinopathies, combined deficiencies, inc destruction

What types of anemias are macrocytic?

megaloblastic, liver disease/alcohol, hemoglobinopathies, metabolic disorders, inc destruction

What is the cause of most bleeding seen in the ER?

trauma

Who should you suspect bleeding disorders in?

spontaneous bleeding from multiple sites, delayed bleeding, bleeding into deep tissues

What helps indicate the cause of bleeding?

site of bleeding

Bleeding from what areas indicates a plt d/o?

mucocutaneous petechiae, ecchymoses, epistaxis, GI, GU, vaginal

Bleeding from what indicates thrombocytopenia, or systemic illness?

purpura

Bleeding from where indicates coagulation factor deficiencies?

joints and potential spaces

What causes dec plt production?

aplastic anemia, lymphoma, leukemia, myelofibrosis, drugs

What causes inc plt destruction?

ITP, drug induced, DIC

What are signs of thrombocytopenia?

nonpalpable petechiae, purpura, mucosal bleeding, hemoptysis, hematuria, hematochezia

When are pts at risk of spontaneous bleeding?

plts < 10,000-20,000

*consider plt transfusion

What is an irreversible cause of plt aggregation impairment?

ASA

What is a reversible cause of plt aggregation impairment?

NSAIDS, clopidogrel, ticlopidine

What causes thromobcytosis ( > 500,000)?

inflammatory rxn: malignancy, polycythemia, postsplenectomy

Hepatocytes synthesize all of the coagulation factors and related regulatory proteins except what?

factor VIII and von Willebrand factor

What indicates liver disease > DIC?

prolong PT + hypofibrinogenemia w/ plasma fibrinogen < 100

What is the tx for clinically significant bleeding or a pending invasive procedure?

transfuse PRBC’s, vit K, cryoprecipitate if low fibrinogen, plt transfusion if low

What organ does Tylenol damage in an overdose?

liver

What is one of the most sensitive test to get in a Tylenol overdose?

PT/INR

What labs indicated DIC?

low plt count, fibrinogen

high D-dimer, PT/PTT

What is the only lab that will be inc in chronic DIC?

D-dimer

What are sx of DIC?

purpura fulminans, AMS, cutaneous gangrene, thrombotic purpura, ARF, jaundice, ARDS

What is the tx for DIC?

cryoprecipitate, plts, FFP, vit K, folate

What is Hemophila A (“classic” hemophilia?

deficiency of factor VIII

What is Hemophilia B (Christmas disease)?

deficiency of factor IX

What defines severe hemophilia?

factors levels < 1% of normal -spontaneous bleeding, difficult to control bleeds

What defines moderate hemophilia?

factor levels 1-5% of normal levels -may bleed spontaneously, but most common bleeding is related to trauma

What defines mild hemophilia?

factor levels 5-25% of normal -usually bleed only after trauma, may be unaware or never know they have it

What are sx of hemophilia?

easy bruising, recurrent bleeding into joints/muscles, hemorrhage; FH, bruising out-of-proportion to injuryW

What labs indicate hemophilia?

PT normal, PTT abn

What is the tx for hemophilia?

complete factor replacement (pts usually have the factor w/ them)

if suspicious but not confirmed → FFP or cryoprecipitate until labs come back

What should you AVOID in hemophilia pts until factor replacement is given?

central lines, LP’s, IM injections, invasive procedures

What is the most common congenital bleeding disorder?

Von Willebrand disease

What is Type 1 von W?

partial quantitative dz (75% of cases)W

What is Type 2 von W?

qualitative abnormalitiesW

What is Type 3 von W?

severe and total deficiency of vWF (rare, autosomal recessive)

What are sx of von Willebrand’s?

skin and mucosal bleeding sx (epitaxis, gingival bleeding, bruising, GI, menorrhagia), many unaware until they have a procedure

What should you avoid in pts w/ von W?

meds w/ antiplt affects (ASA, NSAIDs, heparin, select abx)

What is the tx for von W?

Desmopressin (DDAVP)

*unresponsive → plasma derivatives w/ vWF

What is the most distinguishing feature of SCD?

pain crises

What are the sx of SCD?

pain, infection, acute chest syndrome, stroke

What is the tx for SCD?

pain management (Opioids) & hydration

frequent/severe pain → hydroxyurea (HU)

What is the hallmark of thalassemias?

microcytic, hypochromic, hemolytic anemia

What is a-Thalassemia?

carrier -no clinical sx, microcytic RBCs, borerline Hbg

What is b-Thalassemia minor?

thalassemia trait -mild microcytic anemia w/ basophilic stippling; elevated HbA2

What is b-Thalassemia major?

Cooley anemia - severe anemia, begins w/in the first year of life; hepatosplenomegaly, jaundice, requires regular and lifelong blood transfusions

What are the sx of hereditary spherocytosis?

neonatal jaundice, anemia, splenomegaly, periodic jaundice, pigmented gallstones, MOSTLY asx

What is the tx for hereditary spherocytosis?

supportive care, splenectomy controversial

What are RF for autoimmune hemolytic anemia?

viral or resp infections, pregnancy (5x)

What labs are used to dx AHA?

ab titer, direct and indirect, CBC w/ manual diff

What is the tx for AHA?

supportive care, hematology referral

What are sx of TTP?

thrombocytopenia, fever, renal impairment, microangiopathic hemolytic anemia, neurological impairment (CVA, seizures)

What is the MC precipitating event for TTP?

pregnancy

What labs indicate TTP?

severe anemia, thrombocytopenia (< 20,000), schistocytes or helmet cells

What is the tx for TTP?

daily plasma exchange: plasmapheresis, plasma infusion

When is FFP transfusion warranted?

reversal of Warfarin, bleeding w/ multiple coag defects, correction of coag defects, massive transfusion

When is a cryoprecipitate transfusion warranted?

primary role - fibrinogen or vWF replacement

bleeding w/ low fibrinogen, uremia, DIC, unresponsive to DDAVP

What causes a hemolytic transfusion reaction?

recipients abs induce hemolysis of donor’s RBCs

What is the MC transfusion reaction?

Febrile transfusion reaction

What is the tx for febrile transfusion rxn?

pretx w/ Tylenol, usually self-limiting

What is the tx for allergic transfusion rxn?

pretreat w/ benadryl

What is the tx for clinically significant bleeding d/t Warfarin?

stop anticoagulant, give vit K & FFP

What is there a risk of when reversing Warfarin?

recurrent thrombosis

What reverses UFH?

Protamine

*add FFP & pRBC if severe bleeding

What is the tx for bleeding d/t ASA or NSAIDS?

plt transfusion

*ASA may last 4-5 days; NSAID resolves w/in 1 day

What is the reversal agent for Dabigatran (Pradaxa)?

Idarucizumab

What can reverse Eliquis, Betrixaban, Edoxaban, and Xarelto, and heparins?

Andexanet

What can reverse all NOACs and Heparins?

Ciraparantag

Which agents are used for thrombus dissolution?

Fibrinolytics: Streptokinase, Antistreplase, Alteplase, tPA

Which fibrinolytic agents canNOT be administered w/in 12 months of a strep infection?

Streptokinase, Antistreplase

What is commonly administered after Alteplase or tPA?

Heparin

What is most important when administering a fibrinolytic for acute MI?

rapid administration > agent used

What is used to dissolve a DVT or PE?

UFH or LMWH

*CI → vena cava filter

What fibrinolytic is used for ischemic strokes?

Alteplase

What is the tx for allergic and anaphylactic rxn to fibrinolytics?

Diphenhydramine & Methylprednisolone IV

What is the tx for hypotension d/t fibrinolytics?

slow infusion rate & IV crystalloid

How often should Hct be monitored while a pt is receiving fibrinolytics?

q 4-6 hrs

What is the tx for pathologic fxs?

pain relief and restoration of ambulation or function

What is the tx for acute spinal cord compression?

pain control, Rad tx, surgery, or combo based on extent of disease

What is the tx for upper airway obstruction?

immediate airway control -cricothyrotomy, jet insufflation, tracheostomy

What is thet x for hypercalcemia?

IV isotonic saline, Calcitonin SC or IM, glucocorticoids, bisphosphonate

What is the tx for SIADH?

water restriction, furosemide w/ NS for severe cases

What is the tx for adrenal insufficiency?

IV steroids (glucocorticoid and mineralocorticoid)

Hydrocortisone

What is given for chemo related pain?

parenteral opioids, corticosteroids