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SBL - Blood and Lymph Pathology > RBC pathology > Flashcards

RBC pathology Flashcards

1
Q

What are the typical clinical findings of anemia?

A

Pale, weak, malaise, easily fatigued, dyspnea on exertion, and SOB

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2
Q

How quickly are reticulocytes produced?

A

5 days

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3
Q

What are some long-term effects of chronic anemia and hypoxia?

A

fatty changes to liver, myocardium and kidneys

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4
Q

What are the effects of acute blood loss anemia?

A

A shift of interstitial fluid to intravascular – this lowers hematocrit
Increased EPO production
Iron deficiency
Leukocytosis (for significant blood loss)
Reticulocytosis and thrombocytosis (days after blood loss)

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5
Q

What are the key features of all hemolytic anemias?

A

Shortened RBC life span (<120 days)
Elevated EPO
Accumulation of hemoglobin degredation products (unconj. bilirubin)

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6
Q

Hereditary spherocytosis

A 
RBC vulnerable to splenic destruction due to sphere shape 
AD disorder
increased MCHC
*anemia, splenomegaly and jaundice*
aplastic crisis = parvovirus
hemolytic crisis = mono
tx: splenectomy
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7
Q

G6PD deficiency

A

episodic hemolysis caused by oxidative stress – infections, drugs or food
micro pathology = Heinz bodies and bite cells

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8
Q

Sickle cell disease

A

Glu –> Val mutation

hemolytic anemia, microvascular obstructions and ischemic tissue damage leading to a variety of “crises”

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9
Q

SS vaso-occlusive crisis

A

hypoxic injury and infarction that cause pain in affected region
most severe = acute chest crisis (lungs)

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10
Q

SS sequestration crisis

A

massive entrapment of sickle cells leading to splenic enlargment, hypovolemia and sometimes hypovolemic shock

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11
Q

SS aplastic crisis

A

infection of RBC progenitors by parvovirus B19 causing transient cessation of erythropoiesis and sudden worsening of anemia

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12
Q

Hemoglobin C disease

A

mild hemolytic anemia

mechanism similar to SS but hemoglobin C does not sickle as readily as hemoglobin S

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13
Q

Beta-thalassemia major

A

deficient HbA synthesis resulting in microcytic hypochromic RBC with abnormal O2 transport
ineffective erythropoiesis
RBC prone to splenic destruction
HbF markedly elevated
poor clinical discourse without blood transfusion and iron chelation
hepatosplenomegaly due to extramedullary hematopoiesis
Mediterranean, African and Asian descent

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14
Q

Beta-thalassemia minor

A

patients are asymptomatic
if present, anemia is mild
hypocromic and microcytic (looks like iron deficiency anemia)
increased HbA2 but normal HbF
Mediterranean, African and Asian descent

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15
Q

Alpha-thalassemia

A

reduced or absent synthesis of alpha-globin chains
complete abscence is lethal in-utero
hemolysis and ineffective erythropoiesis less severe than beta-thalassemia
newborns = hemoglobin Barts
children and adults = Hbh

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16
Q

paroxysmal nocturnal hemoglobinuria (PNH)

A

acquired mutation of PIGA gene
susceptible to intravascular hemolysis by complement
typically chronic hemolysis without hemoglobinura despite name
prone to thrombosis

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17
Q

Immunohemolytic anemias

A

caused by antibodies against normal RBC constituents or antigens modified by haptens
results in either extravascular hemolysis or uncommonly complement fixation/intravascular hemolysis

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18
Q

hemolytic anemia from trauma to RBC

A

usually occurs in individuals with cardiac valve prostheses (mechanical) and microangiopathic disorders
vascular changes produce shear stresses than injury circulating RBC

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19
Q

megaloblastic anemias

A

caused by folate or cobalamin deficiency leading to impairment of DNA synthesis
macrocytic morphology

20
Q

pernicious anemia

A

type of megaloblastic anemia caused by autoimmune gastritis that impairs production of IF required for B12 uptake
autoreactive T-cells initiates gastric mucosal injury and triggers formation of IF autoantibodies
leukopenia, elevated homocysteine and elevated methymalonic acid

21
Q

B12 deficiency

A

type of megaloblastic anemia caused by impaired absorption of B12 due to loss of pepsin secretion, gastrectomy, ileal resection or certain tapeworms
elevated homocysteine and methymalonic acid

22
Q

Folate deficiency

A

type of megaloblastic anemia caused by insufficient diet

elevated homocysteine but normal MMA

23
Q

porphyria

A

a deficiency in the pigments involved in the formation of heme (porphyrins) which causes anemia and/or splenomegaly
abnormal reaction to sunlight, abnormal hair growth, teeth staining, seizures, hallucinations, and general psychosis
vampires and werewolves

24
Q

iron deficiency anemia

A 
most commonly due to chronic blood loss
morphologically microcytic and hypochromic 
Low HCT
Low Hgb
Low iron
Low ferritin
Low hepcidin
25
Q

Plummer-Vinson syndrome

A

microcytic hypochromic anemia
esophageal webs
atrophic glossitis

26
Q

aplastic anemia

A

syndrome of chronic primary hematopoietic failure and subsequent pancytopenia
causes include aquired, chemical agents, physical agents and inherited
typical anemia sx
reticulocytopenia; NO splenomegaly

27
Q

pure red cell aplasia

A

primary marrow disorder with only RBC precurors affected

causes include thymoma, autoimmune or parvovirus B19

28
Q

PTT measures which clotting pathway?

A

Intrinsic

XII, XI, IX, VIII

29
Q

PT meaures which clotting pathway?

A

Extrinsic

VII

30
Q

Henoch-Schonlein purpura

A

systemic immune disorder characterized by purpuric rash, colicky abdominal pain, polyarthralgia, and acute glomerulonephritis
due to deposition of immune complexes

31
Q

Hereditary hemorrhagic telangiectasia

A

AD disorder caused by mutations of genes that modulate TFG-B signaling
dilated, tortuous blodd vessels
bleeding common under mucous membranes

32
Q

perivascular amyloidosis

A

amyloid light chain amyloidosis

mucocutaneous petechiae

33
Q

chronic immune thrombocytopenic purpura

A

caused by autoantibody (IgG) destruction of platelets
can occur secondarily to SLE, HIV or B-cell neoplasms
tx: splenectomy
micro shows megathrombocytes
sx include petechiae, ecchymosis, mucosal bleeding, and hemorrhages
NO splenomegaly or lymphadenopathy
PT and PTT are normal

34
Q

acute immune thrombocytopenic purpura

A

similar to chronic but mainly a disease of childhood
sx appear 1-2 weeks after a viral illness
resolves spontaneously

35
Q

drug induced thrombocytopenia

A

typically caused by quinine, quinidine, and vancomycin

36
Q

heparin-induced thrombocytopenia

A

causes severe life threatening venous and arterial thromboses

37
Q

HIV-associated thrombocytopenia

A

HIV can infect megakaryocytes since they contain CD4 and CKCR4 receptor and coreceptor
infected megakaryocytes are prone to apoptosis and opsonization by immune complexes or splenic destruction

38
Q

thrombotic thrombocytopenic purpura

A

deficiency in plasma enzyme ADAMTS13
causes thrombotic microangiopathies
widespread organ dysfunction

39
Q

hemolytic-uremic syndrome

A

triggered by E. coli Shiga-like toxin which alters endothelial function and platelete aggregation causing thrombotic microangiopathies
widespread organ dysfunction

40
Q

Bernard-Soulier syndrome

A

deficiency in endothelial platelet membrane complex Ib-IX (vWF receptor)
generally a pediatric disease with giant platelets in peripheral blood
severe bleeding tendency

41
Q

Glanzmann thrombasthenia

A

deficiency in glycoprotein IIb-IIIa which aids in binding platelets to fibrinogen
mild to severe bleeding tendency

42
Q

Hemophilia A

A

deficiency in factor VIII, which normally binds to vWF once in circulation
prolonged PTT with normal PT
easy bruising, massive hemorrhage after trauma, and spontaneous hemorrhage (hemarthroses)
tx: factor VIII infusions

43
Q

von Willebrand disease

A

deficiency in vWF
only types 1 and 3 are clinically detected by prolonged PTT
type 2 is most common

44
Q

Hemophilia B

A

factor IX deficiency
clinically indistinguishable from Hem A
prolonged PTT with normal PT
tx: factor IX infusions

45
Q

Disseminated intravascular coaguation (DIC)

A

acute, subacute or chronic thrombohemorrhagic disorder characterized by excessive activation of coagulation and foration of thrombi in microvasulature
not a primary disease –complication of a variety of disorders
sx include microangiopathic hemolytic anemia, dyspnea, cyanosis, respiratory failure, subacute renal failure, circulatory failure/shock
tx: treat the underlying cause

SBL - Blood and Lymph Pathology (2 decks)

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