Internal Med - Heme Flashcards

1
Q

Acute lymphocytic leukemia (ALL) is MC in what age group

A

Children - MCC of malignancy peak age 3-7

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2
Q

Child + Lymphadenopathy + bone pain + bleeding + fever

A

ALL

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3
Q

Bone marrow with more than 20% blasts in a child with bone pain

A

ALL

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4
Q

60yo Male with fatigue, blood tests demonstrate severe anemia, decreased neutrophils, small, abnormal B lymphocysts with painless cervical lymphadenopathy and hepatosplenomegaly

A

CLL = Chronic lymphocytic leukemia

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5
Q

MC age for CLL

A

Middle age patient; Peak age of 50

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6
Q

DX of CLL on peripheral smear

A

SMUDGE cells on smear + mature lymphocytes

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7
Q

Tx of ALL and CLL

A

ALL = Highly responsive to chemo; Remission >90%

CLL = Tx with observation; if lymphocytes are >100,000 or symptomatic, tx with chemo

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8
Q

Acute Myelogenous leukemia MC in what age group

A

Most common in adults; 65 years

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9
Q

Dx of AML on peripheral smear

A

Auer Rods

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10
Q

Sx of AML

A

Anemia, DOE, dizziness; Splenomegaly may report early saiety; gum infiltration/gingivitis

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11
Q

Dx tests for AML

A

Bone marrow biopsy = definitive

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12
Q

CML dx

A

Philadelphia chromosome (Ph+); Bcr-Abl is the abnormal gene causing the switch of the philadelphia chromosome and the cell to grow out of control

Most have elevated lactate dehydrogenase level and elevated uric acid

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13
Q

AML on peripheral smear

A

Auer rods + blasts in an adult ot

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14
Q

Philadelphia chromosome

A

CML

Translocation of chromosome 9 & 22

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15
Q

Tx of AML and CML

A

AML → combination chemo, bone marrow transplant

CML → Gleevec (imantinib) which makes this a chronic disease state

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16
Q

Normocytic/normochromic or decreased MCV, decreased TIBC with normal or increased ferritin

A

Anemia of chronic dx

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17
Q

Dx of anemia of chronic dz

A

Requires presence of chronic infection, inflammation, or cancer; Microcytic or normocytic anemia

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18
Q

MCC of anemia of CKD

A

Chronic renal failure, anemia resulting from connective tissue disorders; HIV, SLE, Cancer, Cirrhosis, chronic infections

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19
Q

How does anemia of renal failure affect EPO

A

EPO is impaired d/t decrease in both EPO production + bone marrow responsiveness to EPO

Presence of renal insufficiency or failure + decreased EPO levels

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20
Q

Tx of anemia of CKD

A

Tx underlying disorder

Recombinant EPO and iron supplements

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21
Q

MC genetic bleeding disorder

A

Von Wille; Autosomal dominant

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22
Q

How to differentiate VwD from hemophilia

A

In VWD there is lack of hemarthrosism small amounts of superficial bleeding, common to have bleeding with a minor injury or petechiae

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23
Q

What factor affects VwD?

A

Decrease in VW factor and Factor 8

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24
Q

Tx of VwD

A

DDVAP Desmopressin or in excessive bleeding a transfusion concentrated blood clotting factors containing VW factor

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25
Q

What are the lab values associated with Hemophilia A & B

A

Increased PTT and normal plt count and functio

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26
Q

Most specific test for hemophilias

A

Functional assay for factor 8 & 9

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27
Q

Dx of VWD

A

CBC with plt count and coag studies → Normal CBC, normal plt count and increased bleeding time

Normal or prolonged aPTT and normal PT

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28
Q

Tx of hemophilia A & B

A

Replacement of factor 8 & 9; Pts should avoid situations that provoke bleeding; avoid blood thinners and anti platelet drugs

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29
Q

G6PD is a deficiency of

A

X linked enzyme defect

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30
Q

MC population G6PD is seen in

A

African Americans

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31
Q

Drugs that commonly cause hemolysis in pts with G6PD

A

Antimalarias

Analgesice

Sulnonamides

Dapsone

*Fava Beans

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32
Q

Oxidative stress (infection or drugs) + African American Male + Heinz bodies

A

G6PD

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33
Q

What is seen on a peripheral smear in G6PD

A

Heinz bodies and Bite cells on smear

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34
Q

MC procoagulant clotting factor

A

Factor V leiden

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35
Q

How does factor V cause hypercoagability

A

Mutated factor v resistant to breakdown by activated protein c → Hypercoag

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36
Q

Sx and PE findings in a pt with Factor V leiden

A

increased DVT and PE, especially in young patients

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37
Q

Lab findings in Factor V

A

Activated protein C assay → Factor v functional assay and confirm with DNA testing

Normal PT and PTT

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38
Q

Protein C deficiency

A

Autosomal dominant protein c mutation

Vitamin K dependent anticoag liver protein that stimulates fibronolysis and clot lysis

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39
Q

Dx of protein c deficiency

A

Protein C or S functional assay → Decreased protein c or s activity levels

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40
Q

What are pts with protein c deficiency at risk for?

A

Increased risk of skin necrosis while on warfarin

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41
Q

Tx of protein C deficiency

A

Requires heparin and oral anticoagulation for lif

42
Q

Protein S is a vitamin K-dependent glycoprotein that is a cofactor of

A

Protein C

43
Q

Major clinical manifestation of protein S deficiency

A

Venous thromboembolism; VTE → DVT and PEs

44
Q

Protein S deficiency tx

A

Heparin and oral anticoag for life

45
Q

Antiphospholipid syndrome is an autoimune disorder associated with

A

SLE; Characterized by thromboses and recurrent spontaneous abortions

46
Q

What is the MOA of antiphospholipid syndrome

A

Autoantibodies → React against platelet membranes or prothrombin-platelet membrane complex activating endothelial cells and plts → Activates complement mediated thrombosis

47
Q

Dx of Antiphospholipid syndrome

A

Lupus anticoagulant ; Prolonged dilute russel viper venom time (DRVVT) test; prolonged PTT

48
Q

Tx of antiphospholipid syndrome

A

Pts with thrombotic events require high dose IV heparin and then oral anticoags indefinitely

49
Q

ITP is a blood disorder characterized by abnormal

A

Decreased in number of platatlets in blood; Autoimmune antibody reaction to platelets which results in splenic platelet destruction often after an acute infection

50
Q

Sx of ITP

A

Easy bruising, mucosal bleeding, purpura, petechial raches

51
Q

Whats the difference between primary and secondary ITP?

A

Primary → Immune mediated thrombocytopenia without an underlying condition

Secondary → immune mediated thrombocytopenia with an underlying condition (HIV infection)

52
Q

When does ITP clasically present?

A

1-2 weeks after a viral infection

53
Q

What lab values are associated with ITP

A

Isolated thrombocytopenia and otherwise normal CBC and peipheral blood smear

(+) Direct coombs Test

54
Q

Tx of ITP

A

Observation in pts with plts >30,000 and no bleeding

Corticosteroids initial tx for plts with plts <30,000

IVIG for pts with plts <30,000 + contraindication for corticosteroids or are bleeding or have a high risk of bleeding

*Splenectomy second line tx for pts with refractory ITP

55
Q

Lab values seen in iron deficiency

A

Microcytic (decreased MCV) Hypochromic (Decreased MCH)

Increased TIBC and Decreaesd Ferritin

56
Q

MCC of anemia

A

Iron deficiency and usually from blood loss

57
Q

Tx of iron deficiency

A

Ferrous sulfate 325mg TID

Takes 6 weeks to correct; 6 months to replete iron stores; Recheck blood counts every 3 months x 1 year

58
Q

When would you give packed RBC in a pt with iron deficiency anemia?

A

If Hbg was <8

59
Q

Hodgkins disease lymphoma sx

A

Painless lymphadenopathy ; Upper body lymph nodes → neck, axilla, shoulder, chest

Contiguous spread to local lymph nodes

B symptoms → Fever, wt loss, night sweats

60
Q

Painless lymphadenopathy + Reed sternberg + Bimodal age (20/50) + B sx

What is the dx?

A

Hodgkin Lymphoma

61
Q

Dx of Hodgkin lymphoma

A

DXR → mediastinal adenopathy

Reed sternberg cells are pathognomonic → B cell proliferation with biloped or multilobed nucelus

62
Q

Tx of Hodgkin Lymphoma

A

Chemo, radiation, highly curable

63
Q

EBV is associated with which type of lymphoma in 40% of cases?

A

Hodgkin Lymphoma

64
Q

HIV pt + IG sx + painless LAD

A

Non-Hodgkins lymphoma

65
Q

Sx of Non-hodgkin lymphoma

A

>50 yo, increased risk w/ immunosupression (HIV)

B symptoms not common

Peripheral, multiple nodes; non-contigous extranodal spread (GI + Skin = MC)

SX = SOB, intussusception, bowel obstruction, abd masses

66
Q

Tx of Non-hodgkins lymphoma

A

Rituximab, chemo, variable course

*Variable cure rate

67
Q

Cancer of monoclonal plasma cells

A

Multiple myeloma

68
Q

MC tumor of bone/bone marrow in pt >50 yo

A

Multiple myeloma

69
Q

MC presentation of Multiple Myeloma

A

CRAB

Calcium elevation, renal failure, anemia, bone lesions

70
Q

Dx of Multiple Myeloma

A

UA → Bence jones proteins seen

Blood smear → Rouleaux formation (stacked RBCs)

Xrays → Lytic lesions “punched out” lesions of skull, spine, long bones

Serum/Urine electrophoresis → Monoclonal (m protein) spoke

71
Q

Tx of Multiple myeloma

A

Bone marrow transplant → Definitive

Melphalan, steroids, thalidomide, bortezomib

72
Q

Sickle cell anemia is MC in what population

A

African american

73
Q

Sx of sickle cell anemia

A

Pain! Hemolysis, jaundice, splenomegaly, priapism, poor healing, swelling in hands and feet, acute chest syndrome, pigmented gall stones

Sickle-cell shaped RBCs clog capillaries causing organ ischemia (crisis)

74
Q

How do you monitor a pt in sickle cell crisis

A

High retic count → decreases as pt improves

75
Q

Dx of sickle cell anemia

A

Reticulocytosis; Mildly increased WBCs

Blood smear → sickled RBCs, Howell-jolly bodies, target cells

Hbg 8-10; Hct 10-20

Hb SS = Disease

Hb SA = Trait

*Two parents with sickle cell trait have a 1 in 4 chance of having a child with Hb SS disease

76
Q

Tx of sickle cell disease

A

Vaccines prolong survival

Hydroxyurea may decrease frequency of crises

Infection, bone marrow aplasia, lung involvement can develop acutely and can be fatal

Tx→ High flow O2, pain control during crisis, supportive transfusion with Hbg <6

77
Q

Thalassemia is microcytic hypochromic anemia with elevated iron, what are the 3 types

A

Beta thal major → most severe; mediterranean descent ; failure to thrive

Beta thal minor or beta trait → mild anemia ; often misdiagnosed as iron deficient

Alpha thal → chinese and southeast asian

78
Q

How are thalassemias diagnosed>

A

Hemoglobin electrophroesis

Beta thal major → Hemoglobin A2 and F

Beta thal trait → Hemoglobin A2

Alpha thal → Hemoglobin H (H disease) Hemoglobin barts (hydrops fetalis) and hemoglobin A (trait)

79
Q

What population beta thal major or cooleys anemia MC in?

A

Families who intermarry

80
Q

Untreated Beta thal sx

A

Severe anemia with Hb level 3-7

Massive hepatosplenomegaly

81
Q

How do you differentiate between beta thal and iron deficiency anemia?

A

In iron def the RBC will be normal to low

In beta thal → RBC will be high

82
Q

How does thalassemia present on blood smear

A

Many nucleated erythroblasts; target cells; small pale RBCs and punctate and diffuse basophilia

83
Q

When do pts with beta thal become symptomatic?

A

Normal at birth due to presence of fetal HgbF but become symptomatic at 6mo

84
Q

Treatment of alpha thal

A

Mild thal → No tx

Moderate dz → folate, avoid oxidative stress

Severe dz → blood transfusions; weekly if severe; iron chelating agents; splenectomy; bone marrow transplant is definitive

85
Q

Treatment of beta thal

A

Trait aka beta thal minor → No medical care necessary

Major → Blood transfusions weekly if severe; Iron chelating; Splenectomy; Bone marrow transplantation is definitive

86
Q

Decrease in platelts + anemia + schistocytes (RBC fragments) on smear

A

TTP

87
Q

Difference in TTP vs ITP

A

ITP → Chronic

TTP → Acute febrile disease with multiorgan thrombosis

88
Q

MCC of TTP

A

After drugs → Quinidine, cyclosporine & Pregnancy

Inhibition of ADAMTS13

89
Q

SX of TTP

A

Fat RN

MC in adults

Purpura and Fat RN → Fever, anemia, thrombocytopenia, renal failure, neurological symptoms

90
Q

Dx studies for TTP

A

CBC normal but with low platelts

Schistocytes on smear

(-) Coombs test

91
Q

Tx of TTP

A

Steroids, plasmapharesis

92
Q

Hemolytic Uremic Syndrome HUS is caused by

A

Post infection → E.Coli or Shigellia

MC in children

93
Q

Sx of HUS

A

Severe kidney problems

Low plts + Anemia + renal failure

94
Q

HUS MCC

A

E.coli O157:H7 and diarrheal illness in a child

95
Q

Sx of B12 deficiency

A

Glossitis → Smooth beefy tongue

Neurologic sx poor balance, low proprioceptions

96
Q

Dx studies of B12 deficiency

A

Megaloblastic anemia (MCV>100) Hypersegmented neutrophils

97
Q

Pernicious anemia dx

A

Schilling test

98
Q

Folate deficiency MCC

A

Alcoholism

99
Q

Dx of Folate deficiency

A

Decreased folated, Increased MCV (macrocytic anemia) - Looks like B12 but with no neurologic sx

100
Q

Tx of Folate deficiency

A

PO folic acid 1-5mg QD

Avoid ETOH and folic acid antagonists (bactrim, phenytoin, sulfaslazine)

*Eat green leafy veggies, yeast, legumes, fruits, animal proteins