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Flashcards in module 7 hemolytic anemia Deck (74):
1

signs increased RBC production:

RPI >2, inceased IRF, leukocytosis, nucleated RBCs, polychromasia of RBCs, normoblastic erythroid hyperplasia.

2

signs increased RBC destruction

Anemia, schistocytes, spherocytes, positive DAT, decreased haptoglobin andhemopoexin, increased billirubin and urobillinogen, increased serum LD, increased CO.

3

Signs increased IV hemolysis

hemoglobinemia and uria, hemosiderinuria, methemoglobinemia

4

clinical findings HA

jaundice, gallstones, dark or red urine in IV hemolysis, anemia, thinning cortical bone, Extramedullary hematopoeisis, splenomegaly.

5

loss of vertical interactions

cause microvessicles and spherocytes (ankyrin, band 4 protein 4.2)

6

loss of horizontal interactions leads to

poikilocytes (spectrin, actin, protein 4.1, GPC)

7

acanthocyte

spiked membrane, excess in outer layer

8

stomatocyte

looks like kissing lips or coffee bean,

9

lipid composition defects characterized by

acanthocytes

10

Hereditary spherocytosis

deficient in membrane proteins, permeable monovalent cations. Often combined spectrin ankyrin deficiency. Membrane loss causes decreased SA to volume ratio and spherocyte. More permeable to Na, causing pumps to work harder expend more glucose. Can be well compensated. Can have aplastic crisis after viral infection.

11

HS LAB RESULTS

Reticulocyte count > 8%. MCHC increased. MCV decreased. increased billirubin, increased LD.

12

Osmotic fragility test

incubate in varying NaCl solutions. HS hemolyze at higher salt concentrations than normal. Little water can be absorbed before lysis. If < 2% spherocytes, incubate overnight at 37C before addition to NaCl. Increases SA loss and spherocyte formation.

13

Target cells and thalassemia in osmotic fragility test

target cells such as those in thalassemia shift to right because lysis occurs at lower NaCl concentration due to increased SA to volume ratio.

14

Autohemolysis test

incubate cells at 37C. Increased hemolyisis due to membrane chages. corrected by addition of glucose. Autohemolyisis increases in immunue hemoluytic anemia, but glucose does not correct.

15

HS and HE shape acquired

in the circulation, not created with misshape.

16

HE defect

horizontal interactions, such as: defective spetrin chains decrease dimer : tetramer ratio, band 4.1 defect, , abnormal GPC/ band 3.

17

HE clinical findings

hemolysis mild and well compensated. Membrane more rigid, may provide malaria defense.

18

HE lab findings

>25 % elliptocytes. Osmotic fragility positive but not necessary as picture is obvious. Responds well to splenectomy.

19

HPP defect

disintegrate at 45-46C unlike normal at 50C. or >37C for 6 hours. Due to inheritance of both a mutant spectrin and a decreased expression spectrin gene. Disruption membrane, fragmentation, poikilocytosis.

20

HPP clinical and lab finding

exchange transfuion needed. bizarre shapes. Extreme microcytosis 25-55 fL MCV. Osmotic fragility abnormal after incubation and heat. Autohemolysis not corrected by glucose. SPlenectomy.

21

Overhydragted hereitary stomatocytosis

Gain of Na+ which exceeds K+ loss. Water enters cell, causes formation of stomatocyte.

22

dehydrated hereditary spherocytosis

Loss of K+ which exceeds Na gain. water leaves cell, becomes targeted.

23

HSS clinical and lab findings

abnormal cation permeability. mild to moderate anemia, 10-50% stomatocytes. osmotic fragility and autohemolysis increased, partially corrected by glucose.

24

acanthocytosis

increased sphingomyelin secondary to plasma changes. expansion of outer leaflet, decreased deformability. Not due to proteins. Abetalipoproteinemia, spur cell anemia,

25

glucose corrects for autohemolysis test in

HS not IHA.

26

SPUR CELL ANEMIA

type acanthocytosis associated with liver disease.

27

G6PD

first step in Hexose monophosphate shunt for GSH. Therefore responsible for preventing cellular oxidation.

28

Oxidation causes

iron oxidation and sulfahydryl groups of globin. Cannot bind oxygen, heinz bodies. Ion proetins and lipids also need to stay reduced.

29

Bite and Blister cells

G6PD deficiency. Splenic macrophages take bite out of cell. MCV goes down, MCHC goes up. Can cause spherocytes.

30

Detect G6PD deficiency

spectrometry flourescencePCR.

31

Pyruvate Kinase

important in glycolytic pathwy. Deficeincy leads to Echinocytes, target cells, and HJ bodies.

32

Hemolysis in pyruvate kinase often compensated by

2-3 BPG overproduction, decreases oxygen affinity.

33

Micrangiopathy HA (MAHA)

thrombii in circulation. Fibrin deposits in vasculature damages RBC membrane

34

TPP

microclots damage RBCs. Usually follow infection or pregnancy. Similar to HUS.

35

DIC

often from bacterial blood infection, platelets consumed. Clots damage RBCs. Clotting time extended, abnormal bleeding, blood flow impeded to organs. Multiple organ faiilure. Abnormal coagulation tests differentiate it from TTP and HUS.

36

babesia

normally mild anemia like malaria. transmitted by ticks. PCR and serological tests to dif from malaria.

37

complement fixation

recruiting complement by antibodies.

38

IGG

warm reactive

39

IGM

cold reactive

40

Contrast warm and cold acting hemolysis

Splenic macrophages have direct FCrIGG and can EV hemolyze. IGM can be IV through complement, or EV hrough c3b R on macrophages.

41

AlloAb Hem Anemia examples

HDFN, blood transfusions.

42

IGM complement activation

only one molecule of IGG required to activated classical pathway, therefore much more sensitive. 2 in close proximity required for IGG.

43

IGM detection on RBC

antisera can detect IGM on RBC via agglutination in saline. Zeta potential and size of IGG prevents agglutination.

44

Direct Anti human Globulin test (DAT or direct coombs)

Polyspecific AHG is added, agglutination means either the anti IGG or the anti complement antibodies have bridged zeta potential and caused agglutination. Follow with monospecific AHG. If only complement then likely IGM.

45

IAT indirect anti human globulin or indirect coombs

Detects antibody in plasma. Less Ab required. Ab incubated with known antigenic makeup screening cells. Then add AHG positive if agglutination.

46

pathophysiology WAIHA

most auto Ab against Rh antigens. common in CLL, neoplastic syndromes, autoimmune disorders. Most hemolysis EV.

47

Clinical and Lab findings

Splenomegaly common. Positiv DAT, normocytic normochromic. increased LD, billirubin. Decreased haptoglobin. Increased osmotic fragility. Spherocytes, increased RDW compared to HS.

48

Distinguish HS from WAIHA

DAT. Increased RDW.

49

WAIHIA therpay

Alloantibodies make transfusion difficult. Focus is on preventing auto Ab formation: corticosteroids, splenectomy, IVIG \, plamapheresis.

50

Cold Agglutination Syndrome

Mostly IGM against I antigens. Splenomegaly. Often episodic.

51

Raynaud's phenmomena

pain and cyanosis in extremeties due to cold agglutination of IGM.

52

CAS Lab findings

RBC count decreased for hemoglobin, MCV is falsely elevated due to agglutination. Must warm all reagents and bood to 37C. When warm, normocytic normochromic, spherocytes, rolouex, nucleated RBCS. Dat poly and complement positive, IGG negative.

53

Cold agglutinin test

Titer at which cells agglutinate. For benign cold agglutinins 1:50 or less. For CAS 1:1000.

54

CAS therapy

corticosteroids, keep hands warm. Plasma exchange. Splenectomy not effective.

55

Paroxsymal Cold hemoglobinuria

common in children. Generally appears weeks after certain infections, persists month or more. IGG antibody against p antigens. Biphasic, complement fixation. Hemoglobinuria, jaundice, splenomegaly.

56

PCH clinical findings

weak or no DAT to complement. Can be positive for IAT if done at low temperature. HB levels drop following an attack and appear in blood and urine. Classic IV signs and neutropenic shift to left. No therapy, recover after infection.

57

PCH specific test

incubate two tubes one at 37 one at 4C. Then incubate both at 37. Lysis in the 4C tube confirms PCH.

58

Drug dependent and independent antibodies

describes whether the drug presence is required for the antibodies to react.

59

Drug independent Ab mechanism

usually modifies antigens so no longer recognized as self. Drugs can also alter the membrane so that ab can bind non specifically. DAT positive, complement negative.

60

Hemolytic transfusion reaction

Patients alloantibodies destroy donors RBCs. Immediate usually ABO and IGM can be IV. Delayed: previously sensitized but not detectable in lab. usually IGG usually EV.

61

HDNF how it occurs

mother must be previously sensitized to antigen (previous pregnancy or transfusion). Baby must possess antigen. Mother must produce antibodies to this antigen that are capable of crossing placenta. Only IGG can cross placenta.

62

ABO HDNF

An O mother has anti A and Anti B antibodies. A or B blood type in the fetus: IGG can cross placenta and case anemia even in first child,.

63

billirunin in HDNF

mother conjugates and excretes in utero. After birth, remaining maternal antibodies still cause hemolysis. Infant is not efficient at metabolizing, and buildup of unconjugated can cause neurological defects.

64

hdnf CLINICAL LAB FINDINGS

RETICULOCYTES CAN BE AS HIGH AS 60%. CORD BLOOD BILLIRUBIN IS ELEVATED.

65

treatment for HDNF newborns

phototherapy: lowers billirubin in infants born with HDNF. Exchange transfusions: lowers billirubin, removes maternal antibodies, treats anemia.

66

Rh Ig

injection of Rh ijmmunoglobulin prevents maternal immunization.

67

Hemolytic Uremic Syndrome

hemolytic anemia with RBC fragmentation, thrombocytopenia, acute nepropathy renal failure. Seen in children. MOst common cause is from shiga toxin in ecoli. BUN and creatinine levels increased reflecting kidney damage.

68

G6PD deficiency protects against

malaria. Could be more oxidants, early stage parasites more easily phagocytosed.

69

G6PD sequence events pathophys

decreased NADH leads to oxidation of hb, and attachement of heinzbodies to band 3. Splenic macrophages pit and with progressive membrane loss sperocytes are formed. Spherocytes become trapped in the spleen.

70

G6PD hemolysis usually caused by

exposure to oxidant drugs. Fava beans can also trigger. Primaquine (antimalarial).

71

Hereditary non spherocytic anemia

is chronic. well compensated.

72

blister cell

Hb oxidized and on one side of the cell. Indicative of oxidant related HA.

73

Tests for G6PD deficiency

ascorbate cyanide test as screeening, quantitation at 340nm, molecular.

74

PK clinical findings

autohemolysis increased at 48 hours, not corrected by glucose but corrected by ATP. Measure dissapearance of flourescence at 340 NM.