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H&L High Yield > Chapter 14 > Flashcards

Chapter 14 Flashcards

1
Q

Drugs associated with drug induced thrombocytopenia

A

Quinine and quinidine (malaria drugs)

Vancomycin

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2
Q

In G6PD deficiency, oxidative stress may be due to infections, drugs, or _______ and precipitates hemoglobin as ______

A

Fava beans, Heinz bodies (dark inclusions visible with crystal violet/Heinz preparation)

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3
Q

Syndrome associated with iron deficiency anemia characterized by microcytic hypochromic anemia, atrophic glossitis, and esophageal webs

A

Plummer-Vinson syndrome

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4
Q

Thalassemia (general) is anemia due to decrease RBC _____ and _____

A

Production and lifespan

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5
Q

Most common cause of anemia in hospitalized patients

Associated with low serum iron, reduce TIBC, and abundant stored iron

A

Anemia of chronic disease

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6
Q

Desire to eat non-food substances (i.e. ice, clay, dirt) to replenish depleted iron

A

Pica

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7
Q

Excessive absorption of dietary iron due to suppressed hepcidin and in combination with repeat transfusions leading to iron overload

A

Secondary hemochromatosis

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8
Q

Hereditary hemoglobinopathy due to a point mutation at the 6th position in β-globin (glutamate -> valine) that promotes polymerization of deoxygenated hemoglobin

A

Sickle cell anemia/disease (SS, βS2βS)

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9
Q

Syndrome characterized by defective adhesion of platelets to subendothelial matrix
Qualitative platelet disorder
Due to genetic GPIb deficiency
Blood smear shows enlarged platelets and mild thrombocytopenia

A

Bernard-Soulier syndrome

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10
Q

Bleeding disorder associated with microangiopathic hemolytic anemia and thrombocytopenia but is distinguished by the absence of neurologic symptoms, prominence of acute renal failure, and its frequent occurrence in kids

A

Hemolytic Uremic Syndrome (HUS)

Distinguished from TTP by lack of neuro symptoms and more sever renal failure

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11
Q

Typical hemolytic uremic syndrome (HUS) presents with ______ due to ______ from undercooked ______

A

Bloody diarrhea; E. coli O157:H7; beef

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12
Q

Systemic hypersensitivity due to immune complex deposition (IgA) particularly in the glomerular mesangial region
Presents with purpuric rash, colicky abdominal pain, polyarthralgia, and acute glomerulonephritis

A

Henoch-Schonlein purpura

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13
Q

Immune hemolytic anemia (IHA) subtype:
IgM
Associated with infection (e.g. M. pneumonia, EBV, infectious mononucleosis)
Intravascular hemolysis

A

Cold agglutinin type of IHA

Cold weather is Miserable (IgM)

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14
Q

Sickle cell anemia can present with hyposthenuria

What is this?

A

Inability to concentrate urine

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15
Q

Heparin induced thrombocytopenia (HIT) type I or II:
Occurs rapidly after onset of therapy
Can resolve despite continuing therapy

A

Type I HIT

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16
Q

What is hemarthrosis and what does it present in?

A

Bleeding into weight-bearing joints

Seen in clotting factor abnormalities (not thrombocytopenia)

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17
Q

In sickle cell anemia, decreased MCHC levels cause a milder disease like in _______

A

Homo HbS patients with α-thalassemia

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18
Q

Prolonged PTT or PT in hemophilia A and B

A

Prolonged PTT (normal PT)

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19
Q

Hepcidin regulates iron absorption in the _________

A

Proximal duodenum

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20
Q

Factor IX deficiency

X-linked recessive

A 
Hemophilia B (Christmas disease)
Clinically identical to hemophilia A
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21
Q

_____ is one of the most common hematologists manifestations of HIV infection

A

Thyombocytopenia (such as in HIV-associated thrombocytopenia)

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22
Q

Diagnostically significant finding of iron deficiency

A

Disappearance of stainable iron form macrophages in the bone marrow

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23
Q

Sickle cell patients have increased risk of infection with ____, _____, and _____

A

S. typhi osteomyelitis, S. pneumoniae, and H. influenza

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24
Q

Bleeding disorder characterized by auto-antibodies against platelets
Insidious onset characterized by bleeding into the skin and mucosal surfaces
Most commonly in women (3X as likely as men) <40
No attendant splenomegaly

A

Chronic immune thrombocytopenia purpura (ITP)

Acute ITP is a disease of childhood appearing after viral infection

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25
Q

Prolonged PT or PTT in Vitamin K deficiency

A

Prolonged PT (remember you check OT with warfarin/Coumadin)

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26
Q

____ is often beneficial for patients with extravascular hemolysis

A

Splenectomy - a lot of RBC destruction happens in the spleen
(Extravascular hemolysis involves RBC destruction by the reticuloendothelial system, i.e. macrophages of the spleen, liver, and lymph nodes)

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27
Q

What do Howell-Jolly bodies (residual RNA) indicate?

A

Splenic dysfunction/splenectomy

28
Q

In chronic renal failure leading to anemia, the anemia is proportional to _____

A

The severity of the uremia

29
Q

Most common cause of myelophthisic anemia (marrow failure where space occupying lesions replace normal marrow elements, can resemble spent phase of myeloproliferative disorders)

A

Metastatic cancer from breast, lung, or prostate

30
Q

Is ineffective erythropoiesis more sever in α or β thalassemia?

A

β-thalassemia

31
Q

Thalassemia indicated by high HbA2 (α2δ2)

A

β-thalassemia (minor/trait)

32
Q

Autosomal dominant disorder of TGFβ signaling dysfunction
Bleeding, most commonly mucous membranes of nose, tongue, mouth, eyes, and GI tract
Most frequent symptom/presentation - recurrent epistaxis

A

Hereditary hemorrhagic telangiectasia (Weber-Osler-Rendu syndrome)

33
Q

Immune hemolytic anemia (IHA) subtype:
IgG
Associated with SLE (MCC) and CLL
Associated with penicillins and cephalosporins (induce production of antibodies agains RBC Rh antigens)
Extravascular hemolysis (antibodies act as opsonins)

A

Warm antibody type of IHA (shows warm agglutinin)

warm weather is Great (IgG)

34
Q

Most common cause of primary polycythemia

A

Polycythemia Vera

35
Q

Autosomal recessive deficiency/dysfunction of glycoprotein IIb/IIIa causing defective platelet aggregation

A

Glanzmann thrombasthenia (double n in Glanzmann, double g in aggregation)

36
Q

Decreased ADAMTS13 (due to acquired auto-antibodies against it or mutation) is found in _____

A

Thrombotic thrombocytopenia purpura (TTP)

37
Q

What test is used to measure Von Willebrand factor?

A

Ristocetin agglutination test

38
Q

Treatment for sickle cell anemia and its effect

A

Hydroxyurea (DNA synthesis inhibitor) - increases HbF and has an anti-inflammatory effect

39
Q

Anemia where autoimmune gastritis impairs production of intrinsic factor (IF) that is required for vitamin B12 absorption in the gut

A

Pernicious anemia

40
Q

Primary marrow disorder in which only erythroid progenitors are suppressed

A

Pure red cell aplasia

41
Q

Thalassemia indicated by high HbF (α2γ2)

A

β-thalassemia (major)

42
Q

Leading cause of disease-related death in paroxysmal nocturnal hemoglobinuria (PNH)

A

Venous thrombosis of hepatic, portal, or cerebral veins

43
Q

Most common cause of death in children with sickle cell anemia

A

H. influenza (can cause septicemia and meningitis)

44
Q

Microangiopathic Hemolytic Anemia is most commonly seen with ___

A

DIC

Also in TTP and HUS

45
Q

α-thalassemia gene deletion possibilities and clinical outcomes

A

1 allele deleted - asymptomatic
2 alleles deleted - mild anemia with increased RBC count
3 alleles deleted - HbH tetramers, severe anemia (tissue hypoxia disproportionate to the level of Hb)
4 alleles deleted - Hb Barts, lethal in utero (hydrops fetalis), lifelong dependence on transfusions (iron overload risk)

46
Q

Heparin induced thrombocytopenia (HIT) type I or II:
Life threatening venous and arterial thrombosis
DVT -> PE
Therapy MUST be discontinued and replaced by another anti-clotting agent

A

Type II HIT

47
Q 
Inherited disorder (autosomal dominant) due to intrinsic defects of the RBC membrane skeleton
Morphology - small, dark-staining (hyperchromic) red cells lacking central zone of pallor
RBCs are more vulnerable to splenic sequestration and destruction
A

Hereditary spherocytosis (HS)

48
Q

What diseases have an increased PTT?

A

vWF disease, hemophilia A and B, antiphospholipid syndrome

49
Q

Paroxysmal nocturnal hemoglobinuria (PNH) treatment

A

Eculizumab (prevents C5 conversion to c5a)

50
Q

Paroxysmal nocturnal hemoglobinuria (PNH) is deficient in which 3 GPI-linked proteins that regulate complement activity?

A

CD55 (DAF), CD59, and C8 binding protein

51
Q

Iron deficiency is the most common nutritional disorder in the world (dietary lack, impaired absorption, increased requirement)
What is iron deficiency most commonly due to in the western world?
Iron deficiency in men or postmenopausal women is _____ until proven otherwise (cancer or occult)

A

Chronic blood loss (external hemorrhage or bleeding into the GI or GU tracts)
GI bleed

52
Q

In β-thalassemia, RBC life span is diminished due to ______

A

Imbalance of α and β globin synthesis

53
Q

What is the only hemolytic anemia caused by an acquired genetic defect?
What is the gene and chromosome?

A

Paroxysmal nocturnal hemoglobinuria (PNH)

PIGA gene is X-linked and subject to lionization

54
Q

Most common inherited bleeding disorder of humans (1% of US adult population)

A

Von Willebrand Disease

55
Q

In α-thalassemia, a 2 allele deletion can be cis or trans. Which is seen in Asians vs African Americans? Which is associated with increased risk of severe thalassemia in offspring?

A

Cis - Asian, increased risk of sever thalassemia in offspring, commonly symptomatic
Trans - African American, commonly asymptomatic

56
Q

Pentad of symptoms found in thrombotic thrombocytopenia purpura (TTP)

A 
Fever
Mental changes/transient neurological deficits
Renal failure
Thrombocytopenia
Microangiopathic hemolytic anemia
57
Q

Most common cause of death in patients with sickle cell anemia
What does it present with?

A

Acute chest syndrome (vasoocclusive crisis of the lungs in sick cell patients)
Presents with fever, cough, chest pain, and pulmonary infiltrates

58
Q

PT vs PTT

A 
Prothrombin time (PT) assesses extrinsic pathway (primary pathway in vivo), prolonged due to dysfunction/deficiency of factor VII, measured in patients taking Coumadin
Partial thromboplastin time (PTT) - assesses intrinsic pathway, prolonged due to dysfunction/deficiency in factors VIII, IX, XI, and/or XII, measured in patients taking heparin
59
Q

H. influenzas and Y. enterocolitica require ___ for pathogenicity

A

Iron

60
Q

Syndrome of primary hematopoietic failure and attendant pancytopenia
Associated with chloramphenicol and hepatitis D and E

A

Aplastic anemia

61
Q

Glutamic acid replaced by lysine describes what disease?

What is seen on blood smear?

A

HbSC disease

Characteristic HbC crystals seen in RBCs

62
Q

Sickle cell trait (SA, α2βS2) provides protection against _____

A

Falciparum malaria

63
Q

Most severe mutation in hemophilia A

A

X chromosome inversion (no synthesis)

64
Q

Anemia with the following:
Target cells (Hb collects in center of cell)
Basophilic stippling (indicator of toxic injury to RBCs)
Massive erythroid hyperplasia (crewcut appearance, chipmunk facies)

A

β-thalassemia

65
Q

Acute vs chronic DIC presentation

A

Acute DIC - associated with obstetric complication or major trauma, dominated by bleeding diathesis
Chronic DIC - associated with carcinomatosis, tends to present with thrombotic complications

66
Q 
Factor VIII (essential cofactor for factor IX in coagulation cascade) deficiency
Most common hereditary disease associated with life-threatening bleeding
A

Hemophilia A

H&L High Yield (3 decks)

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