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step II > amboss 6/25 > Flashcards

amboss 6/25 Flashcards

1
Q

what are some hereditary causes of hyper coagulability

A

factor V leiden, protein C def, protein S def, antithrombin III def, hyperhomocysteinemia, plasminogen def, sickle cell anemia

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2
Q

what are some acquired causes of hypercoagulability

A

pregnancy, age, smoking, obesity, surgery, immobilization, trauma, malignancy, nephrotic syndrome oral contraceptive use, SLE, heparin-induced thrombocytopenia

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3
Q

what is the result of heparin treatment

A

increases the PTT; X inhibitor

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4
Q

what is the differential in someone that is treated with heparin, but their PTT doesnt change

A

this means that they are heparin resistant. Usually antithrombin III, high levels of VIII or fibrinogen

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5
Q

what does the PTT measure; which factors

A

VII, XI, IX, VIII, X V, II, I

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6
Q

what would you expect to find on peripheral smear from someone with sickle cell anemia

A

holly-jolly bodies

sickled cells

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7
Q

what are people with sickle cell trait at risk of developing

A

renal papillary necrosis

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8
Q

what GI disorder are people with sickle cell disease at risk for

A

cholelithiasis

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9
Q

what are the most common presentation of people with sickle cell trait

A

hyposthenuria (lots of urine with low osmolarity due to kidney dysfunction). painless hematuria as well. UTIs, chronic kidney disease, renal medullary carcinoma.

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10
Q

what is the best treatment for sickle cell

A

hydroxurea becuase it reduces the incidence of vasoocclusive crises

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11
Q

what are the mechanisms of hydroxyurea treatment for sickle cell

A

increases reticulocyte and fetal hemoglobin

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12
Q

what is the presentation of paroxysmal nocturnal hemaglobinuria

A

intermittent abdominal pain, jaundice, hemaglobin in the urine, intravascular hemolysis with normal blood smear and combs test because this is complement mediated, not antibody. they will have dark urine in the morning

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13
Q

what does paroxysmal nocturnal hemaglobinuria put the patient at highest risk for

A

thrombosis; this is the most common cause of death

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14
Q

what is the presentation of intravascular hemolysis

A

increased LDH, reticulocytes, hemoglobinuria, and decreased haptoglobin

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15
Q

What is the cause of anemia in an elderly person with a murmur in the right upper field

A

aortic stenosis causing shearing forces on the RBC

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16
Q

what is the cause of thrombocytopenia in cirrhosis

A

The main cause is cirrhosis causes portal hypertension and decreased venous drainage from the spleen. This causes hypersplenism and sequestration of upwards of 90% of platelets in the spleen.
also, due to liver dysfunction there is a paucity of hormones (thrombopoeitin) for the production of platelets

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17
Q

what is the presentation of chronic myelogenous leukemia

A

fatigue, splenomegaly and SEVERE leukocytosis (250,000)

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18
Q

what is the presentation of acute myelogenous leukemia

A

fatigue and splenomegaly with leukocytosis (80,000), with Auer rods (cytoplasmic inclusion needles)

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19
Q

what is the significance of 9:22 translocation

A

this is the Philadelphia chromosome BCR:ABL acute lymphocytic leukemia.

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20
Q

what is the first line therapy for acute myelogenous leukemia

A

imatinib

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21
Q

what is the definitive diagnostic test for leukemia

A

bone marrow biopsy; a blood smear is not definitive

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22
Q

what are Down syndrome patients at risk of developing for blood cancers

A

acute lymphoblastic leukemia, acute myeloid leukemia

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23
Q

what is the best way to prevent tumor lysis syndrome

A

IV hydration

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24
Q

presentation of pediatric HUS

A

This child presents with thrombocytopenia, signs of hemolysis (normocytic anemia, ↑ LDH, indirect hyperbilirubinemia, schistocytes), and renal dysfunction (hematuria, proteinuria, ↑ BUN, and ↑ creatinine), which is a triad indicative of hemolytic uremic syndrome (HUS).

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25
Q

most likely cause of pediatric HUS

A

E. coli (STEC) is the most common cause of pediatric HUS (90% of cases)

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26
Q

what is the treatment fro HUS

A

hemodialysis, supportive care.

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27
Q

what are the indications for hemodialysis for hUS

A

oliguria/anuria, azotemia, hyperkalemia, and acidosis. This patient with acidosis and oliguria should be started on hemodialysis in addition to supportive

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28
Q

is plasma exchange used in HUS

A

rarely. it is not very effective, but can be used in refractory cases.

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29
Q

what is the triad of HUS

A

thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury

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30
Q

what is the best prophylactic for malaria in a pregnant woman

A

mefloquine

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31
Q

what is the staining for acute myeloid leukemia

A

CD33/34 and myeloperoxidase

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32
Q

what antibody do we test for in antiphospholipid

A

anticardiolipin; lupus anticoagulant

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33
Q

what is the presentation and treatment for acute pyogenic lymphadenitis

A

usually a swollen, tender, progressively enlarging lymph node on the side of the neck. treat with clindamycin

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34
Q

what is the presentation of splenic sequestration crisis

A

REMEMBER YOUNG CHILD; PRIOR TO AUTOSPLENECTOMY. reticulocyte count increases, hypovolemia, acute onset severe anemia.

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35
Q

what is the presentation of immune thrombocytopenia

A

isolated thrombocytopenia caused by IgG against platelets. women of child bearing age. bleeding from the gums, petechia and purpura.

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36
Q

do we give platelet transfusions to patients with ITP

A

no. they are rapidly destroyed upon infusion

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37
Q

what is the treatment for ITP

A

usually observation and they typically spontaneously resolve within 6 months

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38
Q

does bleeding occur in thrombocyopenia

A

rarely. even with severe <30,000.

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39
Q

what are the treatments for ITP

A

corticosteroids, IVIG, and splenectomy

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40
Q

what is the presentation of hemophilia

A

normal bleeding time, normal platelets, history of easy bruising and bleeding into joint spaces. bleeding with surgeries. typically a family history of bleeding disorders

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41
Q

who is affected by hemophilia

A

males

42
Q

what parameter is prolonged in hemophilia

A

PTT

43
Q

what is the presentation of vonwillebrands

A

increased PTT (can be normal), increased bleeding time. inherited autosomal dominant

44
Q

what is given for warfarin reversal for emergent surgery

A

vitamin K and fresh frozen plasma

45
Q

what is the most specific test for hereditary spherocytpsis

A

Eosin-5-meleimide test.

46
Q

what tests are there for hereditary spherocytosis

A

eosin-5-meleimide test, osmotic fragility test

47
Q

what are the therapies for hereditary spherocuytosis

A

transfusions and phototherapy for acute hemolysis, folic acid supplementation, splenectomy

48
Q

what is a side effect of TMP-SMX

A

agranulocytosis. neutropenia/neutropenic fever

49
Q

what is the most serious side effect of carbamazepine usage

A

agranulocytosis and aplastic anemia

50
Q

what is the treatment for beta thalassemia minor

A

nothing

51
Q

what is beta thalassemia minor

A

Minor variant (heterozygous): unremarkable symptoms (low risk of hemolysis, rarely splenomegaly)

52
Q

what is beta thalassemia major

A

Absence of beta-globin. Major variant (homozygous)
Severe hemolytic anemia that often requires transfusions → secondary iron overload due to hemolysis, transfusion, or both → secondary hemochromatosis
Hepatosplenomegaly
Growth retardation
Skeletal deformities (high forehead, prominent zygomatic bones, and maxilla)
Transient aplastic crisis (secondary to infection with parvovirus B19)
usually presents in childhood.

53
Q

what is alpha thalassemia silent carrier

A

asymptomatic

54
Q

what is alpha thalassemia trait

A

mild hemolytic anemia with normal RBC and RDW

55
Q

what is hemoglobin H disease

A

Hemoglobin H disease
Jaundice and anemia at birth
Chronic hemolytic anemia that may require transfusions → secondary iron overload due to hemolysis, transfusion, or both → secondary hemochromatosis
Hepatosplenomegaly
Skeletal deformities (less common)
Compared to thalassemia beta, symptoms in adults are generally less severe.

56
Q

what is HbBarts

A

Hb-Bart’s hydrops fetalis syndrome (most severe variant of alpha thalassemia)
Intrauterine ascites and hydrops fetalis, severe hepatosplenomegaly, and often cardiac and skeletal anomalies
Incompatible with life (death in utero or shortly after birth)

57
Q

what is alpha thalassemia minima

A

silent carrier with normal HbA, A2, HbF, and HbH and HbBarts are absent.

58
Q

what is alpha thalassemia minor

A

this is trait. decreased HbA, A2, and HbF, increased HbH, but absent HbBarts

59
Q

what is alpha thalassemia intermediate t

A

HbH disease. two alleles of HbA and A2 as well as HbF greatly diminished. two HbH and one Hb Barts

60
Q

what is Hb Barts disease

A

this is major. HbA and A2 as well as HbF absent. two HbH and two Hb Barts

61
Q

when do sickle cell patients undergo asplenia

A

by the age of 4

62
Q

what empiric treatment should be given to sickle cell patients who present with fever and possible sepsis (over the age of 4)

A

broad spectrum antibiotics such as ceftriaxone for coverage of gram positive and negative bacteria
staph, strep, salmonella and e coli are all common in sickle cell disease

63
Q

what are patients with spherocytosis at risk for and what can reduce this risk

A

gallstones. cholecystitis.

splenectomy

64
Q

what are patients with thalassemia at risk for

A

skeletal deformities

65
Q

when do you expect antibiotics to cause anemia in G6PD deficiency

A

within 2-3 days

66
Q

what is the hallmark of spherocytosis

A

increased RDW and MCHC you asshole!

67
Q

what is the hallmark of spherocytosis

A

increased RDW and MCHC you asshole!!

68
Q

what is the hallmark of spherocytosis

A

increased RDW and MCHC you asshole!!!

69
Q

what is the underlying cause of hereditary spherocytosis

A

defective spectrin on the RBC surface

70
Q

what are the labs for iron deficiency anemia

A

decreased iron, decreased ferritin, increased TIBC and RDW, low MCV,

71
Q

what are the staining for acute lymphoblastic leukemia

A

terminal deoxynucleotidyl transferase TdT, CD10, CD19

72
Q

what is the most frequent malignant disease in children and how does it presnet

A

Acute lymphoblastic leukemia (ALL) is the most frequent malignant disease in children and has a peak incidence between the ages of 2–5 years. It manifests with bone pain, fatigue, fever, hepatosplenomegaly, anemia, thrombocytopenia (with bleeding diathesis), and leukocytosis.

73
Q

what cell type is responsible for ALL

A

B cell precursors

74
Q

what should you think when facing bleeding disorder in a woman

A

von willebrand

75
Q

what is the treatment for von willebrand

A

desmopressin

76
Q

what is the mechanism of action for von willebrands treatment with desmopressin

A

increased release from endothelial cell

77
Q

what can maternal diabetes do to the babies hematologic system

A

cause polycythemia/hyperviscosity and infarction

78
Q

what should be supplemented in spherocytosis

A

folic acid

79
Q

what does folic acid deficiency look like

A

glossitis, low reticulocyte count, macrocytic anemia

80
Q

what is the reason that OCPs cause DVTs

A

estrogen in the pill decreases protein S levels, which typically inihibit pro coagulable factors

81
Q

what do protein C and S do? what is the consequence if they do not function

A

inihibit pro coagulable factors

thrombus formation

82
Q

what is the underlying cause of G6PD

A

absence of reduced glutathione

83
Q

what is the protein responsible for spherocytosis

A

ankryin

84
Q

In what leukemia are auer rods the most common

A

acute promyelocytic leukemia

85
Q

what is the treatment for acute promyelocytic leukemia

A

all-trans retinoic acid

86
Q

what is a characteristic finding for multiple myeloma

A

rouleaux formation

87
Q

what is a characteristic finding on histology for chronic lymphocytic leukemia

A

smudge cells

88
Q

what is the most common presentation of CLL

A

generalized lymphadenopathy and splenomegaly in a patient that is over 65.
most common cause of generalized lymphadenopathy

89
Q

what is the cause of hypercalcemia in Hodgkin lymphoma

A

increased 1a-hydroxylase activity AKA increased active vitamin D. The patient will have hypercalcemia with LOW PTH, which is not seen in humoral PrTH production.

90
Q

reed sternberg cells stain how and are pathopneumonic for what

A

CD15/30 positive

hodgkin lymphom a

91
Q

what is tartrate resistant acid phosphatase imply

A

hairy cell leukemia

92
Q

what are two treatments for hairy cell leukemia

A

cladribine and pentostatin

93
Q

what do steroids do to lymphocytes, eosinophils, monocytes and neutrophils

A

lymphopenia, eosinopenia, decrease in monocyte count, and an increase in neutrophils because it inhibits apoptosis

94
Q

what is a low alkaline phosphatase score associated with

A

CML. it is the only cancer that has a low ALKpoH score

95
Q

what is myelodysplastic syndrome and what is it highly associated with

A

highly associated with acute myelocytic leukemia
A group of conditions caused by bone marrow failure. Cytopenias (e.g., thrombocytopenia, anemia) are the hallmark. The etiology may be genetic, environmental (e.g., benzene exposure), and/or iatrogenic (e.g., post-radiation myelodysplasia).

96
Q

what is the presentation for pure red cell aplasia

A

there will no reticulocytes or very low

97
Q

what is the presentation of beta thalassemia minor

A

can present with an isolated microcytic anemia but will be asymptomatic. If there are symptoms look elsewhere

98
Q

what is the presentation of isoniazid treatment

A

there will be a microcytic anemia with normal iron levels

99
Q

what is the treatment for sickle cell stroke

A

exchange transfusion

100
Q

do we give tPA to sickle cell patients

A

no. this is ineffective for sickle cell stroke nd can cause hemorrhage. exchange transfusion protocols are the most effective

step II (22 decks)

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