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EOR - Internal Med > Internal Med - Heme > Flashcards

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
What are the lab values associated with Hemophilia A & B
Increased PTT and normal plt count and functio
26
Most specific test for hemophilias
Functional assay for factor 8 & 9
27
Dx of VWD
CBC with plt count and coag studies → Normal CBC, normal plt count and increased bleeding time Normal or prolonged aPTT and normal PT
28
Tx of hemophilia A & B
Replacement of factor 8 & 9; Pts should avoid situations that provoke bleeding; avoid blood thinners and anti platelet drugs
29
G6PD is a deficiency of
X linked enzyme defect
30
MC population G6PD is seen in
African Americans
31
Drugs that commonly cause hemolysis in pts with G6PD
Antimalarias Analgesice Sulnonamides Dapsone \*Fava Beans
32
Oxidative stress (infection or drugs) + African American Male + Heinz bodies
G6PD
33
What is seen on a peripheral smear in G6PD
Heinz bodies and Bite cells on smear
34
MC procoagulant clotting factor
Factor V leiden
35
How does factor V cause hypercoagability
Mutated factor v resistant to breakdown by activated protein c → Hypercoag
36
Sx and PE findings in a pt with Factor V leiden
increased DVT and PE, especially in young patients
37
Lab findings in Factor V
Activated protein C assay → Factor v functional assay and confirm with DNA testing Normal PT and PTT
38
Protein C deficiency
Autosomal dominant protein c mutation Vitamin K dependent anticoag liver protein that stimulates fibronolysis and clot lysis
39
Dx of protein c deficiency
Protein C or S functional assay → Decreased protein c or s activity levels
40
What are pts with protein c deficiency at risk for?
Increased risk of skin necrosis while on warfarin
41
Tx of protein C deficiency
Requires heparin and oral anticoagulation for lif
42
Protein S is a vitamin K-dependent glycoprotein that is a cofactor of
Protein C
43
Major clinical manifestation of protein S deficiency
Venous thromboembolism; VTE → DVT and PEs
44
Protein S deficiency tx
Heparin and oral anticoag for life
45
Antiphospholipid syndrome is an autoimune disorder associated with
SLE; Characterized by thromboses and recurrent spontaneous abortions
46
What is the MOA of antiphospholipid syndrome
Autoantibodies → React against platelet membranes or prothrombin-platelet membrane complex activating endothelial cells and plts → Activates complement mediated thrombosis
47
Dx of Antiphospholipid syndrome
Lupus anticoagulant ; Prolonged dilute russel viper venom time (DRVVT) test; prolonged PTT
48
Tx of antiphospholipid syndrome
Pts with thrombotic events require high dose IV heparin and then oral anticoags indefinitely
49
ITP is a blood disorder characterized by abnormal
Decreased in number of platatlets in blood; Autoimmune antibody reaction to platelets which results in splenic platelet destruction often after an acute infection
50
Sx of ITP
Easy bruising, mucosal bleeding, purpura, petechial raches
51
Whats the difference between primary and secondary ITP?
Primary → Immune mediated thrombocytopenia without an underlying condition Secondary → immune mediated thrombocytopenia with an underlying condition (HIV infection)
52
When does ITP clasically present?
1-2 weeks after a viral infection
53
What lab values are associated with ITP
Isolated thrombocytopenia and otherwise normal CBC and peipheral blood smear (+) Direct coombs Test
54
Tx of ITP
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
Lab values seen in iron deficiency
Microcytic (decreased MCV) Hypochromic (Decreased MCH) Increased TIBC and Decreaesd Ferritin
56
MCC of anemia
Iron deficiency and usually from blood loss
57
Tx of iron deficiency
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
When would you give packed RBC in a pt with iron deficiency anemia?
If Hbg was \<8
59
Hodgkins disease lymphoma sx
Painless lymphadenopathy ; Upper body lymph nodes → neck, axilla, shoulder, chest Contiguous spread to local lymph nodes B symptoms → Fever, wt loss, night sweats
60
Painless lymphadenopathy + Reed sternberg + Bimodal age (20/50) + B sx What is the dx?
Hodgkin Lymphoma
61
Dx of Hodgkin lymphoma
DXR → mediastinal adenopathy **Reed sternberg cells** are pathognomonic → B cell proliferation with biloped or multilobed nucelus
62
Tx of Hodgkin Lymphoma
Chemo, radiation, highly curable
63
EBV is associated with which type of lymphoma in 40% of cases?
Hodgkin Lymphoma
64
HIV pt + IG sx + painless LAD
Non-Hodgkins lymphoma
65
Sx of Non-hodgkin lymphoma
\>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
Tx of Non-hodgkins lymphoma
Rituximab, chemo, variable course \*Variable cure rate
67
Cancer of monoclonal plasma cells
Multiple myeloma
68
MC tumor of bone/bone marrow in pt \>50 yo
Multiple myeloma
69
MC presentation of Multiple Myeloma
CRAB Calcium elevation, renal failure, anemia, bone lesions
70
Dx of Multiple Myeloma
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
Tx of Multiple myeloma
Bone marrow transplant → Definitive Melphalan, steroids, thalidomide, bortezomib
72
Sickle cell anemia is MC in what population
African american
73
Sx of sickle cell anemia
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
How do you monitor a pt in sickle cell crisis
High retic count → decreases as pt improves
75
Dx of sickle cell anemia
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
Tx of sickle cell disease
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
Thalassemia is microcytic hypochromic anemia with elevated iron, what are the 3 types
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
How are thalassemias diagnosed\>
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
What population beta thal major or cooleys anemia MC in?
Families who intermarry
80
Untreated Beta thal sx
Severe anemia with Hb level 3-7 Massive hepatosplenomegaly
81
How do you differentiate between beta thal and iron deficiency anemia?
In iron def the RBC will be normal to low In beta thal → RBC will be high
82
How does thalassemia present on blood smear
Many nucleated erythroblasts; target cells; small pale RBCs and punctate and diffuse basophilia
83
When do pts with beta thal become symptomatic?
Normal at birth due to presence of fetal HgbF but become symptomatic at 6mo
84
Treatment of alpha thal
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
Treatment of beta thal
Trait aka beta thal minor → No medical care necessary Major → Blood transfusions weekly if severe; Iron chelating; Splenectomy; Bone marrow transplantation is definitive
86
Decrease in platelts + anemia + schistocytes (RBC fragments) on smear
TTP
87
Difference in TTP vs ITP
ITP → Chronic TTP → Acute febrile disease with multiorgan thrombosis
88
MCC of TTP
After drugs → Quinidine, cyclosporine & Pregnancy Inhibition of ADAMTS13
89
SX of TTP
Fat RN MC in adults Purpura and Fat RN → Fever, anemia, thrombocytopenia, renal failure, neurological symptoms
90
Dx studies for TTP
CBC normal but with low platelts Schistocytes on smear (-) Coombs test
91
Tx of TTP
Steroids, plasmapharesis
92
Hemolytic Uremic Syndrome HUS is caused by
Post infection → E.Coli or Shigellia MC in children
93
Sx of HUS
Severe kidney problems Low plts + Anemia + renal failure
94
HUS MCC
E.coli O157:H7 and diarrheal illness in a child
95
Sx of B12 deficiency
Glossitis → Smooth beefy tongue Neurologic sx poor balance, low proprioceptions
96
Dx studies of B12 deficiency
Megaloblastic anemia (MCV\>100) Hypersegmented neutrophils
97
Pernicious anemia dx
Schilling test
98
Folate deficiency MCC
Alcoholism
99
Dx of Folate deficiency
Decreased folated, Increased MCV (macrocytic anemia) - Looks like B12 but with no neurologic sx
100
Tx of Folate deficiency
PO folic acid 1-5mg QD Avoid ETOH and folic acid antagonists (bactrim, phenytoin, sulfaslazine) \*Eat green leafy veggies, yeast, legumes, fruits, animal proteins

EOR - Internal Med (9 decks)

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