Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

First aid > heme > Flashcards

heme Flashcards

1
Q

Fetal erythropoiesis

A 
Fetal Erythropoiesis occurs in :
Yolk sac (3-8 weeks)
Liver (6 weeks to birth)
Spleen (10-28 weeks)
Bone marrow (18 weeks to adults)

Young liver synthesizes Blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Hemoglobin development

A 
Embryonic globins (zeta and epsilon)
Fetal hemoglobin (HbF)= a2y2
Adult hemoglobin (HbA2)= a2B2
Adult hemoglobin (HbA2)= a2d2 (rare)
HbF has a higher affinity for O2 due to less avid binding of 2-3BPG (2-3 BPG tells hemoglobin to release O2), so having less 2-3BPG makes fetal Hgb hold on to O2 more

At birth Hgb is about 50/50 HbF and HbA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

ABO and Rh classification

A

A group has the Anti B antibody (IgM) to B Ag, has the A antigen on it
B group has the Anti A antibody (IgM)
AB group has no antibodies
O group has both antibodies, and they are in IgM and IgG

Rh classification

+ (has the Ag)

Rh - no Ag, so has Anti D antibody (an IgG) (give moms IgG during and after each pregnancy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Hemolytic disease of the newborn AKA erythroblastosis fetalis

A

An Rh - mom with an Rh+ baby
First pregnancy: mom exposed to fetal blood (dutring delivery)-> formation of maternal anti-D IgG. Subsequent pregnancy- antiD IgG crosses the placenta and attacks the Fetal RBCs–> hemolysis in the fetus. Hydrops fetalis, jaundice, kernicterius. Prevent by administrating antiD IgG to Rh - moms during third trimenster and peripartum if fetus turns our +. Prevents maternal anti-D IgG production

ABO hemolytic disease of the newborn- Type O mom type A or B fetus, pre exisiting maternal anti-A or anti B cross IgG antibodies cross placenta–> hemolysis in the fetus. Mild jaundice in the neonate in 24 hours, can occur in the first born, Treatment: phototherapy or exchange transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Hematopoiesis

A

Multipotent stem cell

Myeloid stem cell: Erythroblasts, megakaryocytes, Granulocytes (Eosinophils, Basophils, Bands->PMNs), Monocytes–> Macrophage

Lymphoid cells–> B cells, T cells (Th cells, T cytotoxic ) NK cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Neutrophils

A 
Acute inflammatory response cells. 
Numbers increase in bacterial infections
Phagocytic. 
Multilobed nucleus
Specific granules contain leukocyte alk phos, collagenase, lysozyme, and lactoferrin

Azurophilic granules (lysosomes) contain proteinases, acid phosphastase, myeloperoxidase, B- glucurondase

increased bands= CML or bacterial infections
chemotactics: c5a, IL8 LtB4, bacterial products, kallikirein, platelet activating facotr

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

RBCs

A

Lifespan 120 days
Source of energy is glucose (90% used in glycolysis, 10% used in HMP shunt)
Membranes contain CL/HCO3- antiporter, which allow RBCs to export HCO3 and transport CO2 from the periphery to the lungs for elimination
Anisocytosis- varying sizes
Poikilocytosis = varying shapes

Blue color= RNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Thrombocytes

A

lifespan 8-10 days
When activated by endothelial injury, aggregate with other platelets and interact with fibrinogen to form platelt plug

Contain dense granules (Ca++, ADP, Serotonin, Histamine CASH) and a granules (vWF, Fibrinogen, fibronectin, platelt factor 4)

Approximately 1/2 of platet pool is stored in the spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Macrophages

A

Long life in tissues, differentiate from monocytes
Activated by y-inferon

APCs MHC2
Important cellular component of granulomas

CD14 receptor is what initiates septic shock

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Eosinophils

A

Defend against helminths via MBPs
Bilobed nucleus, Packed with large eosinophilic granules of uniform size

produse histaminases, MBP, eosinophil peroxidase, eosinophil cationic protein

PACCMAN causes of eosinophilia
Parasites
Asthma 
Eosinophilic granulomatosis with polyaniitis (churg Strauss syndrome)
Chronic adrenal insufficiency
Myeloproliferative disorders
Allergic processes
Neoplasia (hodgkin lymphoma)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Basophils

A

Mediate allergic reaction
Densely basophilic granuls

CML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Mast cells

A

Can bind the Fc portion of IgE to membrane
Activated by tissue trauma, C3a and C5a, Surface IgE (cross linking by Ag)

release of histamine, heparin, tryptase, and eosinophil chemotactic factors

Involved in type 1 HS reactions, cromolyn sodium prevents mast cell degranulation, used for asthma prophylaxis

Vancomycin, opioids and radiocontrast dye elicit IgE independent mast cell degranulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Natural killer cells

A

important in innate immunity, especially against intracellular pathogens, larger than B and T cells, with distinctive cytoplasmic lytic granules (containing perforin and granzyme) act on target cells to induce apoptosis

Contain CD56, and CD16

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

B cells

A

Follicles of lymph nodes, white pulp of spleen, unencapsulated lymphoid tissue

Contain Cd19, Cd20 and CD 21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

T cells

A

CD3

CD28 necessary for T cells (Treg, CD4 and CD8)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Plasma cells

A

Large amounts of antibody

Clock face chromatin distribution with nucleas off to the side, RER and Golgi for secratory white part next to nucleus

mostly found in the bone marrow

Multiple myeloma is a plasma cell dyscrasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Primary hemostasis

A

Formation of a platelet plug (endothelial cells contain in the Wieble Palade bodies- vWF and Pselectin with factor 8) they also contain thromboplastin and tPA and PGI2

  1. Injury to the endothelium–> transient vasoconstriction via neural stimulation reflex and endothelin (from damaged cell)
  2. Exposure: vWf binds to exposed collagen
  3. Adhesion: platlets bind vWF bia Gp1b receptor at site of injury–> platelets undergo confirmational change–> Platelets release ADP(allows for exposure of Gp2b3a), TXA2 ( via COX chemotactic for platelets) and Ca+ (needed for coagulation cascade)
  4. Activation ADP binding to P2Y12 receptor induces Gp2b/3a expression on platelet surface
  5. Aggregation: Fibrinogen binds Gp2b3a receptos and links platelets
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Pro vs anti aggregation factors

A

PRO coag: TXA2 (released by platelets), decreased blood flow, increased platelet aggregation

Anti coag: PgI2, NO (from endothelial cell) increased blood flow, decreased platelet aggregation

Temporary plug stop bleeding, unstableunstable and easily dislodged–> coag cascade is secondary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Thrombogenesis

A

formation of insoluble fibrin mesh

ASA irreversibly inhibits COX 1 and 2, thereby inhibiting TXA2 synthesis (no chemotactic)

Clopidorgrel, prasugrel, ticlodipine inhibit ADP induced expression of Gp2b 3a by irreversible blocking of the P2y12 receptor

Abciximab, Eptifibatide, and tirofiban inhibit Gp2b3a (so no fibrinogen binding, FIB or CIX)

Ristocetin is an assay activates vWF to bind Gp1b, failure of aggregation with ristocetin occurs in vWdisease, and Bernard Soulier syndrome

vWF carries/protects factor 8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Bernard soulier syndrome

A

deficiency in Gp1b receptor (no VwF ability to bind)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Kinin cascade

A

Kaikrein activates bradykinin

increases vasodilation, increase permeability, increase in pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Intrinsic pathway

A

12-12a, 11-11a, 9-9a

vWF activates 8– 8a
then 10- 10a

C1 esterase inhibits kalikrein and 12 and 11

C1 esterase inhbitiro dificeincy - hereditary angioedema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Extrinsic path

A

7-7a

Tissue factor activates factor 10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Combined pathway

A

10- 10a via extrinsic and intrinsic path

5a-2-2a (thrombin)

2a- 1 -1a (fibrinogen)- fibrin monomers stabiliizes fibrin between platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Fibrinolytic system

A

tissue Plasminogen activator activates Plasmin which degrades fibrin (D-dimer)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Vitamin K

A

Factors 2, 7 9 and 10 C and S

Epoxide reductase activates Vitamin K which yCarboxylates 2 7 9 10 C S

Warfarin and liver failure inhibits the epoxide reductase

Factor 7 has the shortes half life, factor 2 has the longest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Antithrombin

A

inhibits thrombin factor 2a and factors 7 9 10

heparin induces antithrombin (inhbitition of factor 2 and 10)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Factor 5 leidin

A

produces factor 5 that is resistent to inhibition by activated protein C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Microcytic anemia

A

hemoglobin issues (TAIL)

Defective globin production: Thalessemias
Defective heme synthesis: Anemia of chronic disease, iron deficiency (late), Lead poisoning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Reticulocyte index

A

the corrected reticulocyte count

reticulocyte percent x actual Hct/normal Hct (45%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Iron deficiency anemia

A

low iron due to chronic bleeding (GI loss, menorrhagia), malnutrition, absorption disorders, GI surgery, or increased demand (pregnancy) decreased final step in heme synthesis (Ferrochelatase cant add iron to protoporphyrin in the mitochondira)

labs: decreased iron, increased TIBC, decreased ferritin (stores), increesed free erythrocyte protoporphyrin, increased RDW ( you have some small and some normal size), decreased Index

Symptoms: fatigue, pallor, pica, spoon nails
low hemoglobin->low myoglobin
May manifest as glottitis, cheilosis, Plummer vinson syndrome (triad of iron deficiency anemia, esophageal webs and dysphagia- you dont eat steak bc of webs, then low iron)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Iron transport

A

iron is consumed, absorption occurs in duodenum via DMT1 transporters, the heme form is more readily absorbed from animals

Enterocytes transport the iron across the cell membrane into blood via ferroportin

Transferrin transports the iron in the blood and delivers it to liver and bone marrow for macrophage storage

Storred intracellular iron is bound to ferritin, preventing iron from producing free radicals

Serum iron is iron in the blood
TIBC- measure of transferrin (when iron stores are depleted- you send out more scavengers)
percent saturation- normal=33%
Serum ferritin- reflects iron stores in macrophages and the liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

alpha thalessemia

A

a-globin gene deletion, there are 4 alpha genes on chromosome 16–> decreased alpha globin synethesis. cis deletio (same chromosome, happens in asians)
Trans on different chromosomes (pravelent in africans

normal is aa/aa
aa/a- athal minor, no anemia
a-/a- (trans) , or aa/- - (cis) a thal minor, mild microcytic, hypochromic anemia, cis deletion my worsen outcome for carriers offspring)

a-/ - - hemoglobin H disease (HbH) ecess B globin, forms tetraterms, moderate to severe microcytic hypochromic anemia

  • -/ - - (hemoglobin barts disease, excess y globin gene, hedrops fetalis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

B-thalassemia

A

Point mutations in splice sites and promoter sequences on chromosome 11–> B globin synthesis

Pravelent in meditteraneans

B-thal minor (heterozygote): b chain is underproduced, usually asymptomatic, diagnosed by increased Hgb A2 on electrophoresis
Major (homozygoete- B chain is absent, severe microcytic hypochromic anemia with target cells and increased anisopoikilocytosis (increased RDW) require blood transfusion – 2’ hemochromatosis), marrow expansion (crew cut on x ray, skeletal deformaties, chipmunk facies, extramedularry hepatopoiesis–> hepatosplenomagaly

increased risk of parvovirus

HbS/B thal heterozygote

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Lead poisoning

A

lead inhibits ferrochelatase and ALAD–> decreased heme synthesis and increased RBC protoporphoryn, also inhibits rRNA degradation –> RBCs retain aggregates of rRNA (basophilic stippling), encephalopathy and erythrocyte basophiling abdominal colic and sideroblastic anemia, drops, wrist and foot drop

Dimercaprol and EDTA are 1st line of treatment
Succicmer used for chelation in kids

Lead lines on gingivae and on metaphysis of long bones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Sideroblastic anemia

A

genetic(X linked) defect in ALAS, acquired (myelodysplastic syndromes, and reversible alcohol is the most common lead poisoning, vitamin B6 deficiency, copper deficiency , drugs, linezolid isoniazid

Labs will show: increased iron, normal/low TIBC, increased ferritin, ringed sideroblasts, basophilic stippling of RBCs,

Treatment: pyridoxine (B6 cofactor for ALAS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

MEgaloblastic anemia

A

Impaired DNA synthesis–> malnutrition of nucleus of precursor cells in bone marrow delayer relatice to maturation of cytoplasm

Causes: vitB12 deficiency, folate deficiency, meds (hydroxyurea, phenytoin, methotrexate, sulfa drugs

RBC macrocytosis, hypersegmented neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Folate deficiency

A

causes: malnutrition (alcoholic), malabsorption, drugs (methotrexate, TMP phyenytoin) increased requirement (hemolytic anemia, pregnancy)

increased homocystein, normal methylmalonic acid–> no neuro symptoms (vs B12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

folate metabolism

A

Folate circulates in the serum as methyl-THF, you need to remove methyl group to have DNA synthesis

Vit B12 takes the methyl group, homocysteine takes the methyl group from B12–>methionin

To make homocysteine you need methylmalonic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Vit B12 deficiency

A

causes pernicious anemia, malabsorption (Crohns disease), pancreatic insufficiency gastrectomy, insufficient intake (vegans), Diphyllobothrium latum (fish tapeworm)

increased homocysteine (cant take the methyl group), increased methylmalonic acid

Neuro symptoms- reversible dementia, subacute combined degeneration (due to involvement of B12 in FA pathways and myelin synthesis)
Spinocerebellar tract, lateral corticospinal tract, dorsal column dysfunction

Folate supplementation when really a B12 deficiency can correct the anemia, but neuro symptoms worsen
Liver can store B12 for yearns

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Orotic aciduria

A

Inability to convert orotic acid to UMP (denovo pyrimidine syntheis)

Autosomal recessive
Presents as failure to thrive, dev delay, and megaloblastic anemia refractory to folate and B12
No hyperammonemia (vs ornithine transcarbomylase deficiency where increased orotic acid with hyper ammonemia)

Give UMP or uridine triacetate to bypass mutated enzyme

Orotic acid will accumulate in urine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Non megaloblastic anemia

A

No issue with nucleus division so they are appropriately sized
Macrocytic anemia,
Causes- alcoholosm, liver disease

43
Q

Diamond Blackfan anemia

A

congenital pure red cell aplasia, rapid onset within first yeat of life theres an intrinsic defect in erythrocyte progenitor cells

So your red cells are big
increased HgbF but decrease in total Hgb
Short stature, craniofacial abnormalities, upperextremity malformation( third phalanx on thumb

44
Q

Normocytic normochromic anemia

A

either non hemolytic or hemolytic
the hemolytic anemia can be due to intrinsic (the red cell bursts by itself) or extrinsic (red cell is being sheared)
and by the location of hemolysis (intravascular vs extravascular)

hemolysis can lead to increases in LDH, reticulocytes, unconjugated bilirubin, pigmented gallstones, and urobilinogen in the urine

45
Q

Intravascular hemolysis

A

decrease in haptoglobin (eats up the free hemoglobin), increase in schistocytes on blood smear, hemoglobinuria, hemosiderinuria, and urobilinogen in urin

mechanical hemolysis (prosthetic valve), paroxysmal nocturnal hemoglobinuria, microangiopathic hemolytic anemias

46
Q

Extravascular hemolysis

A

macrophages in spleen clear RBCs, spherocytes (hereditary spherocytosis andautoimmun hemolytic anemia, no hemoglobinuria/hemosiderinuria, urobilinogen in urine

47
Q

Anemia of chronic disease

A

inflammation (IL6- increased hepcidin (released by liver, binds to ferroportin on intestinal mucosal cells and macrophages, thus inhibiting iron transport–> decreased release of iron from macrophages and decreased iron absorption from the gut

chronic infections, neoplastic disorders, CKD, autoimmune diseases

low iron, low TIBC, increased ferritin (youre storing away you iron to divert from infection)

can eventually become microcytic

adress the underlying cause of inflammationcna give EPo

48
Q

Aplastic anemia

A

caused by failure or destruction of hematopoietic stem cells due to: radiation and drugs (benzene, chloamphenicol, alkylating agents, antimetabolites)
Viral agents- EBV, HIV, hepatitis viruses
decreased reticulocyte count, EPO is increased

49
Q

Fanconi anemia

A

AR DNA repair defect–> bone marrow failure, normocytosis or macrocytosis

idiopathic immune mediated

50
Q

hereditary spherocytosis

A

autosomal dominant
defect in proteins interacting with RBC membrane (ankyrin, band 3, protein 4.2, spectrin)

Small round RBC with increased MCHC–> oremature removal by spleen
Splenomegaly, aplastic crisis

eosin 5 malerimide binding test, increased in fragility, normal to decreased MCV with abundant of RBcs

splenectomy treats it

51
Q

G6pD deficiency

A

X-linked recessive, G6pd defect–>decreased NADPH–> decreased reduced glutathione–> increased RBC susceptibility to oxidative stress (sulfa drugs, antimalarials, fava beans–> hemolysis
Causes extravascular and intravascular

Back pain, hemoglobinuria a few days after a stress
Heinz bodies and bite cells

52
Q

Pyruvates kindase deficiency

A

AR
Pyruvate kinase defect–> decreased ATP–> rigid RBCs–> extravascular hemolysis

Increases levels of 23 bpg– decreased hemoglobin affinity O2

hemolytic anemia in newborns

53
Q

Paroxysmal nocturnal hemoglobinuria

A

CD34+ hematopoietic stem cell mutation – increased complement mediated intravascular hemolysis, especially at night (acidosis)

Acquired PIGA mutation–> impaired GPI anchor sythesis for decay accelaratingfacotr and membrane in

Red cells protect themselves from complement By having DAF and MIRL (decay accelerating factor, CD55) DAF and Mirl are attached via GPI (glycosylphosphatidyl inositol)

When GPI is mutated- DAF and MIRL float away rendering the RBC susceptible to complement- especially when complement gets activated during acidosis

RBCs WBC and platelets are lysed, intravascualr hemolysis leads to hemoglobinemia and uria, and hemosiderinuria, main cause of death is thrombosis of hepatic , portal, or cerebral veins (from lysed platelets)

Complications include iron deficiency anemia (hemoglobinuria, and AML

54
Q

Sickle cells disease

A

Autosplenectomy- HJ bodies– increased risk of infection
S pneumonia
Salmonella osteomyelitis
Painful dactylitis (sweiiling of distal joints, priapism, acute chest syndrom, sickling in renal medulla –> hematuria

Treatment is hydroxyurea–> increase HbF and hydration

55
Q

HbC

A

glutamic acid to lyCine mutation in B globin
causes extravacular hemolyisi

HbSC (1 mutation of each have less disease)

Homozygotes- hemoglobin crystals inside RBC target cells

56
Q

Autoimmune hemolytic anemia

A

a normocytic anemia that is usually idiopathic and coombs +
2 types
Warm AIHA-chronic anemia in which iGG causes RBC agglutination, seen in SLE and CLL and certain drugs (methyl dopa)

cold agglutination (acute anemia in which IgM and complement causes RBC agglutination upon exposure to cold, --> painful, blue fingers and toes
seen in CLL, mycoplasma pneumoniae infections, Mononucleosis
57
Q

Direct coombs test

A

Anti- IgG antibody, added to pt RBC, anti-IgG bind the RBC IgG and cause agglutinatination

Indirect just measures if there are anitbodies by adding normal RBCs to serum (if there are Ab the cells willa gglutination

58
Q

Microangiopathic hemolytic anemia

A

Shictocytes
Shearing due to intravascular hemolysis

Seen in TTP (Adamsts13 deficiency- vWF doesnt get cleaved) DIC, HUS (verotoxin binds endthelial cells) SLE HELLP syndrome and HTN

59
Q

Macroangiopathic hemolytic anemia

A

prosthetic heart vavles aortic stenosis

60
Q

Hemolytic anemia die to infection

A

increased destruction of RBCs (malaria, babesia)

61
Q

Corticosteroid eosinopenia

A

Cause neutrophilia, despite causing eosinopenia and lymphopenia,
Corticosteroids cause decreased activation of neutrophil adhesion molecules, impairing migration out of the vasculature to sites of inflammation
Corticosteroids sequester eosinophils in lymph nodes and cause apoptosis of lymphocytes

62
Q

Neutrophil left shift

A

increase in neutrophil precursors, band cells and metamyelocytes in peripheral blood

usually seen with neutrophilia in the acute response to infection or inflammation
Called leukoerythroblastic reaction when left shift is seen with immature RBCs

Severe anemia or marrow responce

63
Q

Heme synthesis, porphyrias, and lead poisoning

A

Porphyrias are hereditary or acquired conditions of defective heme synthesis that lead to accumulation of heme precursors, lead inhibits specific enzymes (ferrochelatase and ALAd) leading ot similar conditions

64
Q

Lead poisoning

A

Ferrochelatase and ALAD deficiency

Protoporphyrin and ALA accumulates
Microcytic anemia (basophilic stippling, ringed sideroblasts in bone marrow), GI and kidney disease

Kids- exposure to lead paint-> mental deterioration
Adults: batteries, ammunition–> headache, memory loss, demyelination (peripheral neuropathy)

65
Q

Acute intermittent porphyria

A

prophobilinogen deaminase deficiency
(alos uroporphyringen 1 synthease
Autosomal dominant mutation

Porphobilinogen, ALA accumulates

Symptoms (painful abdomen, port wine colored pee, polyneuropathy, psychological disturbances, precipitated by drugs (cyp 450 inducers, alcohol, starvation)

Treatment- hemin and glucose

66
Q

Porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase deficiency

Uroporphyrin accurmulates (tea colored urine)

blistering cutaneous photosensitivity and hyperpigmentation

Most common porphyria, exacerbated with alcohol consumption
Causes- familial, hepatitis C
treat with phlebotomy, sun avoidance, antimalarials (hydroxychloroquine)

67
Q

Heme synthesis

A

Glycine, succinyl CoA and B6 make ALA (via ALAS RLS, deficiency causes sideroblastic anemia X linked)

ALA-> Porphobilinogen ( ALAD, lead poisoning)
Porphobilinogen-> Hydroxymethyblane (Porphobilinogen deaminase, Acute intermittent porphyria)

Uro-> copro porphynogen 3 (Uro de carboxylase (porphyria cutanea tarda Tea colored urine)

in mito, ferrochelatase and iton and protoporphyrin–> heme

68
Q

iron poisoning

A

cell death from free radicals and peroxidation of membrane lipids

Abdominal pain, vomiting, GI bleeding–> metabolic acidosis with anion gap, and organ damage, leads to carring with GI obstruction

Chelate with deferoxamine, deferasirox, gastric lavage

Chronic hemochromatosis (arthropathy, cirrhosis, cardiomyopathy, diabetes mellitus and skin pigmentations, hypogonadism, phlebotomy or chelations

69
Q

Hemophilia A B or C

A

elevated pTT
C can be autosomal
Hemarthrosis (bleeding into joints, knee easy bruising,, bleeding after trauma or surgery

Treatment: desmopressin and specific factor

70
Q

Bernard Soulier syndrome

A

decreased Gp1b, no platelet to vWF adhesion

Labs: abnormal ristocetin test, large platelets

71
Q

Glanzmann thrombasthenia

A

Defect in aggregation

Decreased Gp2b3a, blood smear shows no platelet clumping

72
Q

Immune thrombocytopenia

A

destruction of platelet in spleen, Anti-Gp2b3a antibodies–> splenic macrophages phagocytose platelets
idiopathic or autoimmune disorders

Labs: increased megakaryocytes in BM, decreased platelet count

Treatment: steroids, IVIG to give something to spleen to heat

73
Q

TTP (thrombotic thrombocytopenic purpura

A

inhibition of ADAMTS13 (a vWF metalloproteas) -> increase large vWF multimers–> increased platelet adhesion and aggregation

Triad of thrombocytopenia (platelets), MAHA (Hgb decreased, schistocytes, increased LDH), acute kidney injury (increased Cr)

Triad + fever and neuro symptoms
PT and PTT

Treatment: plasmapherisis, steroids, rituximab

74
Q

HUS

A

shiga like toxin from EHEC O157H7 infection

Bloody diarhea

75
Q

vWF

A

tratment: desmopressin, which releases vWF stored in endothelium

76
Q

DIC

A

Snake bites, Sepsis (gram negatives), Trauma, Ob compliation, acute pancreatitis, Malignancy, nephrotic syndrome, Transufion

increased Fibrin degradation products (D dimers, Fibrinogen decreased, decreased factors

77
Q

Factor 5 leiden

A

Protein C active site is messed up

DVT, cerbra vein thrombosis, recurrent pregnancy loss

78
Q

prothrombin mutation gene

A

increasedprotduction of prothrombin-> plasma levels and bvenous clots

79
Q

Leukemia vs Lymphoma

A

Leukemia- lympoid, myeloid neoplasma with involvement in bonemarror, tumor cells in peripheral blood

Lymphoma- discrete tumor mass in lymph nots

80
Q

Hodgkins vs non hodgkin

A

Hodgkin: localized single group of nodes with contigupus spread, better prognosis
Reed sternberg cells with attraction of lymph cells
bimodal age distribution, associated with EBV

Non-hodgkin: multiple lymph nodes involved, extranodal involvment common, worse prognosis, B cells or T cells, can occur in kids and adults, HIV and EBV, HTLV

81
Q

Hodgkin lymphoma

A

Reed sternberg cells
CD15+ and CD30+ of B cell origin

2 owl eyesx 15 =30

Nodular sclerosis- most common
lymphocyte rich- best prognosis
mixed- eosinophilia- immunocomp patients
lymphocyte depleted

82
Q

Burkitt lymphoma

A

t18, 14 translocation of c-myc (8) to heavy chain Ig (14)

Starry sku, sheets of lymph cells with interspersed tingible body macrophages, associated with EBV

Jaw lesion in africa, pelivc or abdomen in sporadic form

83
Q

Diffuse large B cell lymphoma

A

Usually older adults

mutation in BCL2, BCL6 most common

84
Q

Follicular lymphoma

A

Addults
t(14, 18)

18 has BCL2, 14 is ig heavy chain
wax and waning lymphadenopathy

85
Q

Mantle cell lymphoma

A

Males, CD5+ (usually on T cells)

t(11,14) cyclin D on 11 and heavy chain (allows cell to go G-> S phase)

Very aggressive, patients present late

86
Q

marginal zone lymphoma

A

t (11, 18) chronic inflammation, Sjogrens syndrome, chronic gastritis (MALToma)

87
Q

Primary central nervous system

A

EBV related associated with HIV and AIDS

often a single, ring enhancing lesion on MRI should be distinguised from toxoplasmosis via CSF

88
Q

Adult T cell lymphoma

A

Caused HTLV associated with IV drug abuse
Adults with cutaneous lesions, common in japan (T cell Tokyo,

Lytic bone lesions, hypercalcemia

89
Q

Mycosis fungoides/ Sezary syndrome

A

Skin patches and plaques (cut T cell lymphoma) characterized by atypical CD4 cells with cerebriform neuclei and intradermal neoplastic cell aggregates (Pautrier microabcess) may prgress to sezary syndrome (t cell leukemia)

90
Q

Plasma cell dyscrasia

A

characterized by monoclonal immunoglobulin (iG) ovverproduction due to plasma cell disordr

SPEP or free light chain with M spike (overproduction of a monoclonal IgG fragment

91
Q

Multiple myeloma

A 
characterized by 
Overproduction of IgG or sometimes IgA
Clinical features: CRAB (for cancer)
hyperCalcemia
Renal involvement
Anemia
Bone lytic lesions (punched out on X ray)--> Back pain

Peripheral smear shows rouleaux formation (increased protein messes up charge–> RBC stacked)
Urinalysis shows Ig Light chains (Bence jones proteinuria with - urine dipstick

monoclonal Bone marrow, intracytoplasmic inclusions containing igG
Complication: increased risk of infection, 1’ amyloidosis (ALight chian)

92
Q

Waldenstrom macroglobinemia

A

Over production of IgM (macroglobinemia because igN is the largest Ig)

Clinical features: peripheral neuropathy, no CRAB findings, hyperviscosity syndrome (Headache, Blurry vision, Raynaud phenomenon, Retinal hemorrhages)

Bone marrow analysis - small lymphocytes with IgM containing vacuoules

Lymphoplasmacytic lymphoma
Complications: thrombosis

93
Q

Monoclonol gammopathy of undetermined significance

A

over production of any Ig type, usually asymptomatic. No CRAB findings,
1-2% risk per year of transitioning to multiple myeloma

94
Q

Myelodysplastic syndromes

A

Stem cell disorders involving ineffective hematopoiesis–> defects in cell maturation of non lymphoid lineages

Caused by denovo mutations or environmental exposure (radiation, benzene, chemotherapy)

Risk of transformation to AML

Pseudo Pelger Huet anomaly- neutrophils with dilobed (duet nuclei, typically seen after chemo)

95
Q

Leukemias-

A

unregulated growth and differentiation of WBCs in bone marrow–> marrow failure-> anemia, infections, and hemorrhage
increased fucked up WBC in blood,

Leukemic cell infiltration of liver, spleen, lymph nodes and skin (leukemia cutis possible)

96
Q

Acute lymphoblastic leukemia/lymphoma

A

usually in kids, Tcell ALL can present with mediastinal mass (SVC-like syndrome)
Associated with down syndrome

Peripheral blood and marrow have an increase in lymphoblasts
TdT+ (marker of pre-T and pre B cell, CD 10 + )marker of pre B cells)

Most responsive to therapy

May spread to CNS and testes

t 12,21–> better brognosis

97
Q

Chronic lymphocytic leukemia/ small lymphocytic lymphoma

A

Older, Most common adult leukemia
CD20+ , CD23+, CD 5+ B cell neoplasms

Often asymptomatic, progresses slowly, smudge cells in peripheral smear, autoimmune hemolytic anemia

CLL= Crushed Little lymphocytes (smudge cells)
Richter transformation CLL SLL

98
Q

Hairy cell leukemia

A

Adult males Mature B cell tumor, Cells have hair projections, peripheral lymphadenopathy is uncommon

Causes marrow fibrosis (dry ttrap on aspirtation

associated with BRAF
Treatment: Cladribine, pentostatin

99
Q

Acute myelogenous leukemia

A 
Auer rods (MPO condensates)
usually acute promyelocytic --> increased circulating myeloblasts on peripheral smear

Risk factors: alkylating chemotherapy, radiation, myeloproliferative disorders, Down syndrom
APL (t15 17) responds to all trans retinoic acid vit A

100
Q

CML

A

Philidelphia chromosome, t 9 22 (BCR-ABL)
Mature and matureing granulocutes
very low Alk Phos (cells not appropriate)

Responds to BCR-ABL tyrosine kinase inhbitiors (Imatinib)

101
Q

Chronic myeloproliferative

A

malignant hematopoietic neoplasms with varying impacts on WBCs and myeloid cell lines

Polycythemia vera (acquired JAK2 mutation)- may present as intense itching after shower, svere burning pain, decreased EPO. Treatment is phelbotomy, hydroxyurea, ruxlininiv (JAK 1/2 inhbitor)

Essential thrombocythemia: characterized by massive proliferation of megakaryocytes, bleeding and thrombosis,

Myelofibrosis: Obliteation of bone marrow with fibrosis (increased fibroblast activity, associated with massive splenomegaly and teardrop,

102
Q

LANGERHANS CELL histiocytosis

A

Collective group of proliferative disorders
Presents un a child as lytic bone lesions and skin rash or as a recuttent otitis media witha mass in the mastoid

Cells are immature and dont activate T cells

Cells express S100 mesodermal origin and CD1a
Bierbech franules (tennis rackets or rod shaped on EM
103
Q

Tumor lysis syndrome

A

Oncologic emergency
Release of K and PO4–
Ca++ gets sequesterd by PO4—
Increased nucleic acid breakdown–> hyperuricemia–> AKI
Prevention via aggressive hydration, allopurinol, rasburicase

First aid (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions