chinese heme

exaggerated immune response to non harmful antigens

hypersensitivity

immediate IgE hypersensitivity reaction

type I

antibody dependent cytotoxic hypersensitivity reaction

type II

immune complex hypersensitivity reaction

type III

delayed T cell hypersensitivity reaction

type IV

allergic reaction, anaphylaxis, hay fever, asthma

type I hypersensitivity

antibodies bind to cell surface antigen causing cellular destruction, inflammation, cellular dysfunction

type II hypersensitivity

transfusion reactions, rheumatic fever

type II hypersensitivity

IgE stimulated by allergen

type I hypersensitivity

IgG antigen antibody immune complex attracts neutrophils which release lysosomal enzymes

type III hypersensitivity

SLE hypersensitivity type

III

no antibody involved, T cell delayed reaction (48 hrs)

type IV hypersensitivity

DM1, graft vs host dz

type IV hypersensitivity

what is anemia

decreased erythrocytes

hemoglobin in anemia

<13.5 g/dL in males

<12.5 in females

symptomatic hemoglobin in anemia

<10

anemia sx

fatigue is first, pallor, HA, angina

best initial lab for anemia

CBC w/ peripheral blood smear

what is the general quantitative relationship between hemoglobin and hematocrit

hematocrit should be ~3x the hemoglobin

increased destruction anemia reticulocytic index

>2% (making more babies to compensate for the big guys that get destroyed)

destruction anemia blood smear

normocytic

reduced production anemia reticulocytic index

<2% (not making enough babies)

production anemia blood smear

micro or macrocytic

nl MCV

80-100

macrocytic anemia MCV

>120

neutrophil hypersegmentation

nl is 3-5 segments, megaloblastic anemia=neutrophils have >5 lobes

what is megaloblastic anemia

neutrophils have >5 lobes

folic acid deficiency causes what kind of anemia

macrocytic and megaloblastic, m/c in pregnancy, tx w/ supplements (folic acid=vit B9)

what kind of anemia does vit B12 deficiency cause

macrocytic and megaloblastic

how do you get vit B12

intrinsic factors from stomach help absorption in ileum

vit B12 deficiency RF

pernicious anemia, Crohn dz, gastric bypass

what causes pernicious anemia

antiparietal cell antibodies and anti intrinsic factor antibodies (parietal cells secrete intrinsic factor)

vit B12 deficiency sx

anemia and peripheral neuropathy

dx vit B12 deficiency

methyl malonic acid (MMA) blood test

Schilling test

vit B12 deficiency methyl malonic acid blood test results

increased

what is vit B12 Schilling test and what do the results mean

admin B12 PO

if + in urine, it’s a nutrient problem

if - in urine, it’s a gastric cause

tx B12 deficiency

B12 PO if it’s a nutrient problem

B12 IV if it’s a gastric problem

non megaloblastic anemia

no hypersegmentation on peripheral blood smear

causes of non megaloblastic macrocytic anemia

liver dz, alcohol/drug use, metabolic syndrome

normocytic anemia MCV

80-100

what labs should you order to determine if there is hemolysis in normocytic anemia

LDH, haptoglobin, unconjugated, UA

what should you check if there is hemolysis

blood smear

hemolysis lab results

increased LDH and bilirubin

decreased haptoglobin

what kind of hemolysis causes + hemoglobin in UA

intra vascular

causes of intravascular hemolysis

DIC (schistocytes)

transfusion rxn

infection (sepsis)

paroxysmal nocturnal hemoglobinuria

IV Rho immune globulin infusion

what kind of hemolysis causes - hemoglobin in UA

G6PD deficiency (bite cells, Heinz bodies)

sickle cell

hereditary spherocytosis

warm-cold autoimmune hemolysis

infection (bartonella, malaria)

what kind of anemia do transfusion reactions cause

normocytic

when is Hb ~7-8

sepsis, cardiac surgery, chemotherapy, heart failure

when is Hb ~8-10

sx anemia, ongoing bleeding, ACS, noncardiac surgery

what transfusion rxns happen in seconds to minutes

anaphylaxis: IgA deficiency and primary HoTN rxn

rapid shock, respiratory distress, angioedema, urticaria seconds to minutes after transfusion

anaphylaxis: IgA deficiency

tx IgA deficiency anaphylaxis

washing

transient HoTN in a pt taking ACEI seconds to minutes after transfusion

anaphylaxis: primary HoTN rxn

what usually causes primary HoTN transfusion rxn

bradykinin in blood product

what transfusion rxns occur in ~1 hour

acute hemolytic: ABO blood type, bacterial sepsis (m/c pseudomonas)

fever, chill, hemoglobinuria, flank pain ~1 hr post transfusion

ABO blood type transfusion rxn

fever, chill, septic shock, DIC ~1 hr post transfusion

acute hemolytic bacterial sepsis

what transfusion rxn occurs ~1-6 hrs post transfusion

febrile nonhemolytic, a transfusion related acute lung injury (TRACLI) cause by donor’s leukocytes released cytokines

respiratory distress and noncardiogenic pulmonary edema 1-6 hrs post transfusion

febrile nonhemolytic transfusion rxn

what transfusion rxn occurs >2 days later

delayed hemolysis

what kind of anemia does G6PD deficiency cause

normocytic

G6PD deficiency pathology

XLR d/o causing a deficiency in G6PD leading to oxidative stress induced hemolysis (sulfa drugs, dapsone, bactrim)

hemoglobinuria and back pain hours after an oxidative stress

G6PD deficiency

G6PD deficiency blood smear

Heinz bodies: abnl accumulation of Hb

bite cells: spleen removes (“bites”) the Heinz body

what kind of anemia does sickle cell cause

normocytic

sickle cell pathology

double beta globin mutation causes abnl Val to replace nl Glu, leading to >90% HbS which polymerizes when deoxygenated, creating sickle cells

severe pain in chest/back/thigh in sickle cell

due to occlusion of microcirculation

what increases the risk of occlusion in sickle cell

dehydration and apoxia/acidosis

what can occlusion cause in sickle cell

ACS, PNA, fat embolism, avascular necrosis, stroke, dactylitis (usually in kids)

chronic sickle cell sx

expansion of hematopoisesis into skull, hepatomegaly

dx sickle cell

sickle cells in blood smear, Hb electrophoresis shows increased HbF (inhibits polymerization of HbS) and HbS

initial sickle cell tx

oxygen, NS hydration, pain meds

tx sickle cell and fever

ceftriaxone, levofloxacin, moxifloxacin

tx sickle cell and +PCR for parvo19

IVIG and transfusion

tx sickle cell and HbS<30%

exchange transfusion

tx sickle cell and proteinuria

ACEI

hydroxyurea indicates

increased HbF in sickle cell

all sickle cell pts should get what ppx

folate supplementation

PNA vaccination

sickle cell blood smear

sickle cells

target cells due to low oxygen and high cell volume

sickle cell disease vs trait

trait has only one beta globin mutation —> <50% HbS

trait is asx

trait blood smear shows no sickle cells or target cells

causes of non hemolytic normocytic anemia

acute blood loss, CKD, pure red cell aplasia, alcoholic liver dz, anemia from inflammation

dx acute blood loss

reticulocytes>100,000

dx CKD

decreased erythropoietin

what causes pure red cell aplasia

pregnancy, thymoma, malignancy

dx pure red cell aplasia

CD57 and large granular lymphocytosis on smear

dx alcohol liver dz

acanthocytes on smear (abnl RBC w/ irregular spiky/thorny appearance)

dx anemia from rheumatoid arthritis inflammation

increased hepcidin

microcytic anemia MCV

<80

first step in microcytic anemia w/u

iron pannel

Hb in iron deficiency anemia

<10

Hb in thalassemia

>10

thalassemia smear

target cell

what kind of anemia is thalassemia

microcytic

alpha thalassemia pathology

gene deletion, normally 4 alpha genes on chromosome 16

alpha thalassemia sx

one alpha gene deletion: asx

two deletions: anemia

three deletions: severe anemia

four deletions: lethal in utero (hydrops fetalis)

beta thalassemia pathology

gene mutation: 2 beta genes on chromosome 11

absent=B⁰

diminished=B⁺

B thalassemia minor

B⁰/B⁺

asx

B thalassemia major

B⁰/B⁰

severe anemia a few months after birth

dx thalassemia

Hb electrophoresis

Hb electrophoresis in alpha thalassemia

nl