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RBC path Flashcards

1
Q

Anemia

A

reduction of total circulating red cell mass below normal limits

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2
Q

Major mechanisms of anemia

A

blood loss, increased red cell destruction, decreased red cell production

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3
Q

Most useful lab values for anemia eval

A

mean cell volume, mean cell hgb, mean cell hgb concentration, RDW

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4
Q

Common clinical findings in anemia

A

pale, weakness, malaise, easy fatigability, dyspnea, SOB; chronic hypoxia can lead to fatty change in liver, myocardium, and kidney

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5
Q

What does HCT measure?

A

relative concentration of red cells, not the total red cell mass

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6
Q

How quickly are reticulocytes produced

A

5 days

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7
Q

Common features of hemolytic anemia

A

shortened red cell life span, elevated EPO levels and compensatory increase in EPO, accumulation of hgb degradation products

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8
Q

Where are senescent RBC destroyed?

A

macrophages in spleen, liver, bone marrow

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9
Q

Extravascular hemolysis

A

caused by alterations that render red cell less deformable, makes it more difficult for cells to go through spleen

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10
Q

Sxs of extravascular hemolysis

A

anemia, splenomegaly, jaundice

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11
Q

Intravascular hemolysis

A

caused by mechanical injury, complement fixation, intracellular parasites, exogenous toxic factors

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12
Q

Manifestations of intravascular hemolysis

A

anemia, hemoglobinemia, hemoglobinuria, hemosidonuria, jaundice

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13
Q

Role of haptoglobin

A

binds free hemoglobin, produces a complex that is cleared by phagocytes

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14
Q

Is bilirubin in uncomplicated hemolytic anemias conjugate or unconjugated?

A

unconjugated

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15
Q

Hereditary Spherocytosis

A

inherited disorder caused by intrinsic defects in red cell membrane skeleton that render red cells spheroid, less deformable, and vulnerable to splenic sequestration and destruction

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16
Q

Inheritance pattern of HS

A

autosomal dominant

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17
Q

Potential proteins affected by HS

A

spectrin a or b, ankyrin, band 3, protein 4.1 and band 4.2

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18
Q

G6PD deficiency

A

abnormalities reduce the ability of red cells to protect themselves from oxidative injury

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19
Q

What may cause episodic hemolysis characteristic of G60D?

A

infections such as viral hepatitis, pneumonia, typhoid fever; drugs; foods

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20
Q

Heinz bodies

A

denatured globin chains that appear as dark inclusions within red cells stained with crystal violet; will be plucked out by macrophages and produce bite cells

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21
Q

When will acute hemolysis begin following exposure of G6PD-deficient individuals to oxidants?

A

2 to 3 days

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22
Q

Most prominent form of Hgb in adults

A

A (alpha 2, beta 2)

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23
Q

Most prominent form of Hgb in newborns

A

HbF

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24
Q

Genetic aberration in sickle cell disease

A

point mutation in B globin, resulting in red cell distortion; glutamate replaced by valine

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25
Q

Vaso-occlusive crises

A

episodes of hypoxic injury and infarction that often cause severe pain

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26
Q

Acute chest syndrome

A

fever, cough, chest pain, pulmonary infiltrates and inflammation

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27
Q

Priapism

A

affects males after puberty and may lead to hypoxic damage and erectile dysfunction

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28
Q

Sequestration crises

A

entrapment of sickled red cells leading to rapid splenic enlargement, hypovolemia, shock

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29
Q

hyposthenuria

A

inability to concentrate urine

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30
Q

What types of organisms are children with sickle cell at increased susceptibility for?

A

encapsulated - S. pneumoniae, H. influenzae meningitis and septicemia

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31
Q

Thalassemia syndromes

A

group of disorders caused by inherited mutations that decrease synthesis of alpha or beta globin chains leading to anemia, tissue hypoxia, and red cell hemolysis

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32
Q

What chromosome codes for alpha globin

A

Chromosome 16

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33
Q

What chromosome codes for beta globin

A

chromosome 11

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34
Q

What do globin defects in thalassemia cause?

A

anemia via decreased red cell production and decreased red cell lifespan

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35
Q

Types of B-Thalassemia mutations

A

B0 mutation (absent synthesis), B+ mutations (reduced synthesis)

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36
Q

Two ways B-thalassemia causes anemia

A

deficit in HbA synthesis produces hypochromic, microcytic red cell; diminished survival of red cells and their precursors

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37
Q

Hemolysis in B-thalassemia and cause

A

extravascular hemolysis due to membrane damage from imbalance of alpha and beta globin chains

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38
Q

Which areas is B-thalassemia major most common in?

A

Mediterranean countries, parts of Africa, and Southeast Asia

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39
Q

Predominant type of Hb in B-thalassemia major individuals

A

HbF

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40
Q

Clinical course of B-thalassemia major

A

brief unless blood transfusions are given, growth retardation, bony prominences, hepatosplenomegaly; cardiac disease resulting from progressive iron overload and secondary hemochromatosis

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41
Q

Morphology of B-Thalassemia major

A

marked variation in size and shape, microcytosis, hypochromia

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42
Q

B-thalassemia minor

A

patients typically asymptomatic but may experience mild anemia

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43
Q

B-thalassemia minor blood smear

A

red cell abnormalities including hypochromia, microcytosis, basophilic stippling and target cells

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44
Q

HbF levels in B-thalassemia minor

A

normal or slightly increased

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45
Q

Why is it important to recognize B-thalassemia minor

A

superficially resembles hypochromic microcytic anemia of iron deficiency, important for genetic counseling

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46
Q

alpha thalassemia caused by…

A

inherited deletions that result in reduced or absent synthesis of a-globin chains

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47
Q

hemoglobin Barts

A

excess unpaired gamma globin chains that form gamma tetramers, seen in newborns with a-thalassemia

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48
Q

HbH

A

excess B-globin chains form tetramers, seen in older children and adults with a-thalassemia

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49
Q

Clinical syndromes of a-thalassemia

A

silent carrier state, a-thalassemia trait, HbH disease, hydrops fetalis

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50
Q

Paroxysmal nocturnal hemoglobinuria

A

acquired mutations in PIGA gene, essential for membrane associated complement regulatory proteins

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51
Q

What type of hemolysis is manifested in PNH?

A

intravascular hemolysis

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52
Q

Leading cause of death in PNH patients

A

thrombosis

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53
Q

CAs developed by PNH syndrome

A

acute myeloid leukemia, myelodysplastic syndrome

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54
Q

What GPI-linked proteins are deficient in patients with PNH?

A

CD55 (decay-accelerating factor), CD59 (membrane inhibitor of reactive lysis), C8 binding protein

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55
Q

Immunohemolytic anemias

A

caused by abs that bind to red cells leading to premature destruction

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56
Q

Warm antibody type immunohemolytic anemia

A

most common form, idiopathic or caused by drugs; IgG abs active at 37C

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57
Q

Cold agglutinin type immunohemolytic anemia

A

IgM abs bind below 37C, appear following an acute infection (Mycoplasma pneumoniae, EBV, CMV, influenza, HIV) or may be chronic

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58
Q

Clinical manifestations of cold agglutinin immunohemolytic anemia

A

pallor, cyanosis, Raynaud phenomenon

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59
Q

Cold hemolysin type immunohemolytic anemia

A

IgG abs bind below 37C, occurs in children following viral infections

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60
Q

Direct Coombs antiglobulin test

A

patient’s red cells mixed with sera containing abs specific for human immunoglobulin or complement, abs will cause agglutination if immunoglobulin or complement is present

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61
Q

Indirect Coombs antiglobulin test

A

patient’s serum tested for ability to agglutinate commercially available red cells bearing defined ags, used to characterize ag target and temp dependence of ab

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62
Q

Most significant hemolysis caused by trauma to red cells is in seen in individuals with…

A

cardiac valve prostheses, microangiopathic disorders, shear forces produced by turbulent blood flow and pressure gradients

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63
Q

Megaloblastic anemia

A

impairment of DNA synthesis leading to ineffective hematopoiesis

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64
Q

Macrocytic changes/morphology

A

peripheral blood has pokilocytic red cells, macrocytic red cells, hypersegmented polys, MCHC “normal,” low reticulocyte count; bone marrow is hypercellular with bands and dysplastic metamyelocytes

65
Q

Neurologic complications of B12 deficiency

A

spastic paraparesis, sensory ataxia, severe paresthesias of lower limb.

66
Q

Pernicious anemia

A

form of megaloblastic anemia caused by autoimmune gastritis that impairs production of IF, required for vitamin B12 uptake

67
Q

Diagnostic values of pernicious anemia

A

moderate to severe megaloblastic anemia, autoantibodies to IF, leukopenia with hypersegmented granulocytes, low serum B12, elevated serum levels of homocysteine and methylmalonic acid

68
Q

Primary cause of gastric pathology in pernicious anemia

A

autoreactive T-cell response initiates gastric mucosal injury and triggers formation of autoantibodies

69
Q

B12 deficiency due to loss of pepsin secretion and achlorydria

A

vitamin B12 not released from proteins in food

70
Q

Vitamin B 12 deficiency due to loss of exocrine pancreatic function

A

Vitamin B12 not released from haptocorrin-vitaminB12 complexes

71
Q

Folic acid deficiency

A

megaloblastic anemia due to suppressed DNA synthesis

72
Q

Homocysteine and MMA levels in folate deficiency

A

homocysteine elevated, MMA normal

73
Q

Homocysteine and MMA levels in vitamin B12 deficiency

A

homocysteine and MMA elevated

74
Q

Porphyrins

A

pigments involved in formation of heme

75
Q

Clinical manifestation of porphyria

A

abnormal reactions to sunlight, staining of teeth, nervous system issues such as referred pain, seizures, hallucinations, and general psychosis

76
Q

Most common nutritional disorder in the world

A

iron deficiency anemia

77
Q

Most common populations to develop IDA

A

toddlers, adolescent girls, women of child bearing age

78
Q

What protein transports iron throughout the body?

A

transferrin

79
Q

What protein stores iron?

A

ferritin

80
Q

Most functional iron in humans is found in what protein?

A

hemoglobin

81
Q

Ferritin levels in IDA

A

<12ug/L

82
Q

Ferritin levels in iron overload

A

5000 ug/L

83
Q

What molecule regulates iron absorption

A

hepcidin

84
Q

Most common cause of iron deficiency in the western world

A

chronic blood loss, like a GI bleed

85
Q

Plummer-Vinson syndrome

A

esophageal webs, microcytic hypochromic anemia, atrophic glossitis

86
Q

Anemia of chronic disease

A

impaired red cell production associated with chronic diseases that produce systemic inflammation

87
Q

How does chronic inflammation suppress erythropoiesis?

A

stimulating hepcidin

88
Q

Ferritin and TIBC in IDA

A

low ferritin, high TIBC

89
Q

Ferritin and TIBC in anemia of chronic disease

A

high ferritin, low TIBC

90
Q

Aplastic anemia

A

exhibits pancytopenia and can be acquired, inherited or triggered by chemical or physical agents

91
Q

Two major etiologies of aplastic anemia

A

extrinsic, immune-mediated suppression of marrow precursors, stem-cell abnormalities

92
Q

Aplastic anemia morphology

A

hypocellular bone marrow, fat cells, fibrous stroma, scattered lymphocytes, plasma cells

93
Q

Clinical features of aplastic anemia

A

pancytopenia, weakness, pallor, dyspnea, thrombocytopenia, neutropenia, reticulopenia

94
Q

Pure red cell aplasia

A

marrow disorder, only red cell precursors are affected

95
Q

Etiology of pure red cell aplasia

A

thymoma, large, granular lymphocytic leukemia, drugs, autoimmune disorders, parvovirus infection

96
Q

Myelophthisic anemia

A

form of marrow failure in which space-occupying lesions replace normal marrow elements most often due to metastatic CAs

97
Q

Polycythemia

A

abnormally high red cell count, increased Hgb

98
Q

Causes of relative polycythemia

A

dehydration, Gaisböck syndrome

99
Q

Absolute polycythemia

A

primary when it results from intrinsic abnormality, secondary when progenitors are responding to a high level of EPO

100
Q

Three main causes of excessive bleeding

A

increased fragility of vessels, platelet deficiency or dysfunction, derangement of coagulation

101
Q

Primary pathway for initiation of coagulation

A

extrinsic (TF) pathway

102
Q

Partial thromboplastin time

A

utilized for detecting abnormalities of blood clotting and to monitor treatment effect of heparin; tests intrinsic and extrinsic pathways; look at factors V, VIII, IX, X, XI, XII, prothrombin, fibrinogen

103
Q

Prothrombin time

A

tests extrinsic and common clotting pathways; coumadin therapy; look at factors V, VII, X, prothrombin, fibrinogen

104
Q

Bleeding disorders caused by vessel wall abnormalities usually present as….

A

small hemorrhages in skin and mucous membranes

105
Q

Clinical conditions that cause abnormalities in vessel walls

A

infections, drug reactions, scurvy and ehlers-danlos, Henoch Schönlein purpura, hereditary hemorrhagic telangiectasia, perivascular amyloidosis

106
Q

Thrombocytopenia

A

count <100,000/uL, <20,000 associated with spontaneous bleeding

107
Q

PT and PTT in thrombocytopenia

A

normal, pathways remain intact

108
Q

Thrombocytopenia due to decreased platelet production

A

results from conditions that depress marrow output or affect megakaryocytes

109
Q

Thrombocytopenia due to decreased platelet survival

A

destruction caused by deposition of abs or immune complexes on platelets, may also occur via mechanical injury

110
Q

Thrombocytopenia due to sequestration

A

if spleen is enlarged, it can harbor 80-90% of platelets

111
Q

Thrombocytopenia due to dilution

A

result from massive transfusions, prolonged blood storage decreases the number of viable platelets

112
Q

Chronic immune thrombocytopenic purpura

A

caused by autoab mediated destruction of platelets, can be idiopathic or secondary

113
Q

Chronic ITP autoantibodies

A

directed against platelet membrane glycoproteins, typically IgG abs

114
Q

Major site of platelet removal

A

spleen

115
Q

Morphological characteristics of chronic ITP

A

spleen is of normal size, marrow has increased megakaryocytes, abnormally large platelets in peripheral blood

116
Q

Clinical features of Chronic ITP

A

adult women <40 yo, bleeding into skin and mucosal surfaces, history of easy bruising or nosebleeds, hemorrages into soft tissues; may first present as melena, hematuria ,excessive menstrual flow, subarachnoid hemorrhage, intracerebral hemorrhage

117
Q

Lab findings of chronic ITP

A

low platelet count, normal or increased megakaryocytes, large platelets in peripheral blood, PT and PTT normal

118
Q

Acute ITP

A

mainly a disease of childhood, appear abruptly 1-2 weeks after a self-limited viral illness; most will self resolve

119
Q

Drug-induced thrombocytopenia

A

most common drugs to cause platelet destruction are quinine, quinidine, and vancomycin

120
Q

Type I heparin-induced thrombocytopenia

A

occurs rapidly after initiation of therapy and generally resolves

121
Q

Type II heparin-induced thrombocytopenia

A

less common, begins 5-14 days after initiation of therapy; results in severe, sometimes life-threatening venous and arterial thromboses

122
Q

HIV-associated thrombocytopenia

A

common manifestation of HIV, HIV-infected megakaryocytes (due to CD4 and CXCR4) making them prone to apoptosis, production of autoantibodies also occurs due to B cell hyperplasia

123
Q

Thrombotic microangiopathy

A

caused by insults that lead to excessive platelet activation and cause the deposition of thrombi in small blood vessels

124
Q

Thrombocytopenic purpura presentation

A

fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neuro deficits and renal failure

125
Q

Hemolytic-Uremic syndrome presentation

A

microangiopathic hemolytic anemia and thrombocytopenia, NO neuro sxs, acute renal failure, frequently occurs in children

126
Q

PT and PTT in thrombotic microangiopathies

A

normal

127
Q

Typical cause of HUS

A

E. coli strain O157:H7

128
Q

Atypical causes of HUS

A

alternative complement pathway inhibitor deficiencies

129
Q

Genetic aberration in TTP

A

deficiency of ADAMTS13

130
Q

Categories of inherited platelet disorders

A

defects of adhesion, aggregation, secretion

131
Q

Bernard-Soulier syndrome

A

deficiency of glycoprotein complex Ib-IX, cannot attach to vWF

132
Q

Glanzmann thronbasthenia

A

failure of platelets to aggregate due to dysfx of glycoprotein IIb-IIIa

133
Q

Causes of disorders of platelet secretion

A

defective release of certain mediators, storage pool disorders

134
Q

Acquired defects of platelet function

A

ingestion of aspirin and other NSAIDs, uremia

135
Q

Common bleeding patterns that occur due to coagulation deficiencies

A

GI and GU tracts, hemarthrosis

136
Q

Hemophilia A deficiency

A

Factor VIII

137
Q

Hemophilia B deficiency

A

Factor IX

138
Q

Vitamin K deficiency results in the impaired synthesis of which factors?

A

II, VII, IX, X, and protein C

139
Q

Major source of factor VIII

A

sinusoidal endothelial cells and kupfer cells

140
Q

Most common inherited bleeding disorder of humans

A

von Willebrand disease

141
Q

Type 1 vW disease

A

autosomal dominant disorder, spectrum of presentations but often mild symptoms

142
Q

Type 2 vW disease

A

autosomal dominant disorder, qualitative defects in vWF, normal number but missense mutations make it defective

143
Q

Type 3 vW disease

A

autosomal recessive disorder, low levels of vWF and severe clinical manifestations

144
Q

PTT in Type 1 and Type 3 vW disease

A

prolonged

145
Q

Inheritance pattern of hemophilia A

A

X-linked recessive

146
Q

Genetic aberration of most severe hemophilia A

A

inversion involve X csome that completely abolishes synthesis of factor VIII

147
Q

Hemophilia A symptoms

A

easy bruising and massive hemorrhage after trauma or operative procedures, hemarthroses, prolonged PTT and normal PT

148
Q

PTT and PT in Hemophilia B

A

prolonged PTT, normal PT

149
Q

Disseminated Intravascular Coagulation

A

acute, subacute, or chronic thrombohemorrhagic disorder characterized by excessive activation of coagulation and formation of thrombi in microvasculature of the body

150
Q

Two major mechanisms that trigger DIC

A

release of tissue factor, widespread injury to endothelial cells

151
Q

Clinical features of DIC

A

most are obstetric patients, microangiopathic hemolytic anemia, dyspnea, cyanosis, respiratory failure, convulsions, coma, oliguria, acute renal failure

152
Q

Sugar residue on A blood antigen

A

N-acetylgalactosamine

153
Q

Sugar residue on B blood antigen

A

galactose

154
Q

Febrile nonhemolytic reaction

A

fever and chills, mild dyspnea, often occurs within 6 hours of a transfusion

155
Q

Allergic rx in blood transfusion

A

urticarial reaction, potentially fatal reactions can occur in pts with IgA deficiency

156
Q

Acute hemolytic reaction - blood transfusions

A

IgM abs against donor red cells that fix complement; fevers, shaking, chills, flank pain rapidly appear; may progress to DIC, shock, acute renal failure, potentially death

157
Q

Delayed hemolytic reaction - blood transfusions

A

typically caused by IgG abs, induce complement activation and cause severe reactions identical to those reacting to ABO mismatches

158
Q

Transfusion-Related Acute Lung Injury

A

severe, frequently fatal complication in which factors trigger the activation of neutrophils in lung microvasculature; dramatic onset resp failure during or soon after transfusion, bilateral pulmonary infiltrates, fever, hypotension, hypoxemia

SBL (21 decks)

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