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Step 2 CK > Hematology > Flashcards

Hematology Flashcards

1
Q

What are sx of anemia based on

A

Severity of disease

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2
Q

Sx w/ HCT >30-35

A

None

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3
Q

Sx w/ HCT 25-30

A

Dyspnea worse on exertion

Fatigue

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4
Q

Sx w/ HCT 20-25

A

Lightheadedness

Angina

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5
Q

Sx w/ HCT

A

Syncope

CP

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6
Q

What kills you in anemia

A

MI

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7
Q

What conditions are indistinguishable for myocytes in the heart

A

Anemia
Hypoxia
CAD
Carbon monoxide poisoning

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8
Q

First clue to etiology of anemia

A

MCV

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9
Q

Causes of low MCV

A

Fe def
Thalassemia
Sideroblastic anemia
AOCD

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10
Q

Only microcytic anemia with elevated retic count

A

Alpha-thalassemia

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11
Q

Causes of high MCV

A 
B12/Folate def
Sideroblastic anemia
Alcohol
Azathioprine, 6-MP, hydroxyurea
Liver disease/hypothyroidism
Zidovudine, phenytoin
MDS
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12
Q

2 main causes of normocytic anemia acutely

A

Blood loss

Hemolysis

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13
Q

Retic count in normocytic anemia

A

Elevated when there is hemolysis

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14
Q

Treatment for severe anemia

A

pRBCs

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15
Q

When do you transfuse anemic pt

A

Symptomatic

HCT 25-30 in elderly pt or one w/ heart disease

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16
Q

What is symptomatic anemia

A

SOB
Lightheadedness, confusion, syncope
Hypotension, tachy
CP

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17
Q

What to transfuse for IgA def pts

A

IgA def donor FFP

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18
Q

HCT of pRBCs

A

70-80%

Whole blood w/ 150mL plasma removed

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19
Q

Effect of pRBC on HCT

A

Each unit raises HCT by 3 points

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20
Q

Who doesn’t get FFP

A

Hemophilia A, B

Von Willebrand

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21
Q

What does FFP replace

A

Clotting factors

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22
Q

What does cryoprecipitate replace

A

Fibrinogen (for DIC)

Has high VIII and VWF

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23
Q

MCV in microcytic anemia

A

Decreased

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24
Q

What is the main reason for Fe def anemia

A

Blood loss

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25
Q

Body needs for Fe

A

Normal - 1-2mg/day
Menstruating - 2-3
Pregnant - 5-6

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26
Q

What is the suspected etiology of AOCD

A

Fe trapped in macrophages or ferritin so Hb synth doesn’t occur

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27
Q

How does renal failure cause AOCD

A

Deficiency of EPO

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28
Q

When is sideroblastic anemia macrocytic

A

MDS

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29
Q

MCC sideroblastic anemia

A

Alcohol

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30
Q

Less common causes of sideroblastic anemia

A

Pb poisoning
Isoniazid
B6 def

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31
Q

Most likely anemia with GI bleeding

A

Fe def

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32
Q

Most likely anemia with menstruation

A

Fe def

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33
Q

Most likely anemia with cancer/chronic infection

A

AOCD

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34
Q

Most likely anemia with RA

A

AOCD

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35
Q

Most likely anemia in alcoholics

A

Sideroblastic

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36
Q

Most likely anemia with Asx pts

A

Thalassemia

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37
Q

Best initial test for microcytic anemia

A

Fe studies

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38
Q

MC microcytic anemia with target cells

A

Thalassemia

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39
Q

Which anemia has low ferritin

A

Fe def

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40
Q

Which anemia has high Fe

A

Sideroblastic

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41
Q

Which anemia has normal Fe studies

A

Thalassemia

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42
Q

Fe def iron studies

A

Low ferritin

High TIBC

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43
Q

AOCD Fe studies

A

High ferritin

Low TIBC

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44
Q

Unique lab features of Fe def

A

Increased RDW

Increased platelets

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45
Q

Single most accurate test for Fe def

A

Bone marrow Bx for stainable Fe

NEVER done

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46
Q

Unique lab features for sideroblastic anemia

A

Prussian blue staining for ringed sideroblasts (most accurate)
Basophilic stippling

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47
Q

Type of anemia in Chronic renal failure

A

Normocytic

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48
Q

Most accurate test for Thalassemia

A

Hb electrophoresis

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49
Q

Most accurate test for alpha-thalassemia

A

Genetic studies

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50
Q

Thalassemia with HbH and increased retics

A

3-del

HbH are beta tetrads

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51
Q

1 gene deletion features of B-thal

A

Increased HbF, HbA2

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52
Q

2 gene deletion features of a-thal

A

Mild anemia

Normal electrophoresis

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53
Q

3 gene deletion features of B-thal

A

Normal HbF

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54
Q

4 gene deletion features of a-thal

A

gamma-4 tetrads (Hb Bart)

CHF causes death in utero

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55
Q

RDW in thalassemia

A

Normal

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56
Q

Rx Fe def

A

Oral ferrous sulfate

IM Fe if insufficient

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57
Q

Rx AOCD

A

Fix underlying problem

Only Anemia from ESRD responds to EPO

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58
Q

Rx Sideroblastic anemia

A

Correct cause

B6/pyridoxine replacement

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59
Q

Rx Thalassemia

A

B - Lifelong chronic transfusions (NOT 3-del)

Fe overload - Deferasirox, deferiprone, defereoxamine

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60
Q

First step in Macrocytic Anemia

A

Peripheral smear

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61
Q

What is macrocytic anemia

A

Presence of hypersegmented neutrophils

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62
Q

What does B12/Folate defiency do that alcoholism cannot

A

Hypersegmented neutrophils

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63
Q

Causes of B12 deficiency

A

Pernicious anemia
Pancreatic insufficiency
Vegan
Crohn’s/Celiac/Sprue/Radiation/Terminal ileum damage
Blind loop syndrome/Gastrectomy/Gastric bypass
Diphyllobothrium latum/HIV

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64
Q

Causes of B9 deficiency

A

Goat’s milk, Celiac
Psoriasis and skin loss/turnover (sickle cell)
Phenytoin, Sulfa
MTX usage in RA

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65
Q

MC neuro abnormality in B12 deficieny

A

Peripheral neuropathy

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66
Q

Classic neuro loss in B12 defiency

A

Posterior column damage (position and vibratory)

AKA Subacute combined degeneration

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67
Q

Which deficiency is associated with increased methylmalonic acid levels

A

B12

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68
Q

LAb values common to B12 and folate deficiency

A 
Megaloblastic anemia
Increased LDH and indirect bili
Decreased retic
Hypercellular marrow
Macroovalocytes
Increased homocysteine
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69
Q

Why is B12 NL in 1/3 of pts with B12 def

A

Carrier protein transcobalamin is acute phase reactant and may be elevated

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70
Q

Confirmatory test for pernicious anemia

A

Anti-intrinsic factor

Anti-parietal cell

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71
Q

Why is retic count low in macrocytic anemia

A

RBCs destroyed as they leave the marrow

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72
Q

Why does pancreatic insufficiency cause macrocytic anemia

A

Pancreatic enzymes are needed to absorb B12

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73
Q

What can result from B12/folate replacement

A

Hyperkalemia

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74
Q

What is common in all hemolysis

A 
Decreased HCT
Increased LDH, indirect Bili, retics
Decreased haptoglobin
NL/Increased MCV
Hyperkalemia
Folate deficiency
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75
Q

What does haptoglobin do

A

Transfers Hb from intravascular to extravascular

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76
Q

What is chronic hemolysis always associated with

A

Bilirubin gallstones

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77
Q

Why is urine dark in hemolytic anemia

A

Increased Hb

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78
Q

What can prolongued hemoglobinuria lead to

A

Renal failure/ATN

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79
Q

MCC hemolysis

A

Sickle cell

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80
Q

Retic count in sickle cell

A

Always High

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81
Q

Precipitants of vasoocclusive crisis

A

Hypoxia
Dehydration/Contrast
Infection/Fever
Cold Temperatures

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82
Q

Common features of sickle cell pts

A

African american

Pain in chest, back, thighs +/- fever

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83
Q

Common manifestations of sickle cell

A 
Bilirubin gallstones
Infection from autosplenectomy
Osteomyelitis
Retinopathy
Stroke
Cardiomegaly
Leg skin ulcers
Avascular necrosis
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84
Q

Best initial test sickle cell

A

Peripheral smear

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85
Q

Most accurate test sickle cell

A

Hb electrophoresis

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86
Q

What is the only Sx in sickle cell trait

A

Hematuria

Isosthenuria

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87
Q

Large volume HCT drop in pt with sickle cell

A

Parvovirus B19
Dx - PCR (best) or IgM
Rx - IVIG

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88
Q

Seen in neutrophils in Ehrlichia infections

A

Morulae

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89
Q

Cause of Howell-Jolly bodies

A

Splenectomy

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90
Q

Rx Sickle cell

A

O2/Hydration/Analgesia
Folate chronically
Pneumococcal vaccine

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91
Q

Prophylaxis for sickle cell crisis

A

Hydroxyurea

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92
Q

Rx if fever/white count in sickle cell

A

Ceftriaxone
Levofloxacin
Moxifloxacin

Don’t wait for culture

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93
Q

When to use exchange transfusion in severe vasoocclusive crisis

A

Acute chest
Priapism
Stroke
Visual disturbance from retinal infarction

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94
Q

What is hereditary spherocytosis

A

Defect in RBC cytoskeleton leading to loss in flexibility

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95
Q

Most common features in hereditary spherocytosis

A 
Recurrent hemolysis
Intermittent jaundice
Splenomegaly
FHx anemia/hemolysis
Bilirubin gallstones
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96
Q

Dx tests hereditary spherocytosis

A

Low MCV
Increased MCHC
Neg Coombs

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97
Q

Most accurate test for hereditary spherocytosis

A

Osmotic fragility

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98
Q

Rx hereditary spherocytosis

A

Folate replacement

Splenectomy

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99
Q

Causes of Autoimmune hemolysis

A 
50% Idiopathic
CLL
Lymphoma
SLE, RA
PCN, alpha-methyldopa, rifampin, phenytoin, quinidine
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100
Q

Most accurate test autoimmune hemolysis

A

Coombs

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101
Q

Best initial therapy autoimmune hemolysis

A

Steroids

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102
Q

Other Rx for autoimmune hemolysis

A

Splenectomy (if reccurent)
IVIG (if not responding to steroids)
Rituximab, azathioprine, cyclophosphamide, cyclosporine (if splenectomy doesn’t control)

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103
Q

Alternative drugs to steroids in autoimmune hemolysis

A

Azathioprine
Cyclophosphamide
Cyclosporine
Mycophenolate mofetil

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104
Q

What is cold agglutinin disease

A

IgM Abs against RBC

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105
Q

What is cold agglutinin disease associated with

A

EBV
Waldenstrom
Mycoplasma pneumoniae

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106
Q

Where does cold agglutinin disease present

A

“Cold” body parts

- Nose, ears, fingers, toes

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107
Q

Most accurate test for cold agglutinin disease

A

Cold agglutinin titre

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108
Q

Rx cold agglutinin disease

A

Stay warm
Rituximab or plasmapheresis
Cyclophosphamide, cyclosporine

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109
Q

What are cryoglobulins associated with

A

Hep C
Joint pain
Glomerulonepheritis

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110
Q

Who gets G6PD def

A

Men (XR)

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111
Q

What is the MCC oxidative stress in G6PD def

A

Infection

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112
Q

Causes of oxidative stress in G6PD def

A 
Dapsone
Quinidine
Sulfa drugs
Primaquine
Nitrofurantoin
Fava beans
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113
Q

MC features in G6PD def

A

African American/Mediterranean
Sudden anemia and jaundice
Normal spleen

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114
Q

Best initial test for G6PD def

A

Heinz bodies and bite cells

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115
Q

Most accurate test for G6PD def

A

G6PD level 1-2mos after hemolytic episode

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116
Q

Stain used for heinz bodies

A

Methylene blue

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117
Q

Rx G6PD def

A

Avoid oxidative stress

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118
Q

Cause of HUS/TTP

A

Deffiency of ADAMTS 13 (metalloproteinase)

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119
Q

HUS associated with

A

Kids

E. coli 0157:H7

120
Q

TTP associated with

A 
Ticlopidine
Clopidogreal
Cyclosporine
AIDS
SLE
Adults - neuro sx and fever
121
Q

Similar features in TTP/HUS

A

Intravascular hemolysis - schistocytes
Thrombocytopenia
Renal insufficiency

122
Q

Rx TTP/HUS

A

Plasmapheresis
FFP if there is delay
NEVER give platelets
Dialysis if BUN > 80

123
Q

What is PNH

A

Clonal stem cell defect increasing RBC sensitivity to complement in acidosis

124
Q

Defect in PNH

A

DAF - CD 55 and 59

Gene - PIG-A

125
Q

Mechanism in PNH

A

↑ resp → ↑pCO2 → ↓pH → Hemolysis + Thrombosis

126
Q

Features of PNH

A

Dark urine with first urination of the day

Pancytopenia and Fe def anemia

127
Q

MCC death in PNH

A

Mesenteric and Hepatic vein Thrombosis

128
Q

Most accurate test for PNH

A

Decreased CD 55+59 (Flow cytometry)

129
Q

Best initial therapy for PNH

A

Predisone

130
Q

Only cure for PNH

A

BM transplant

131
Q

Complement inhibitor for PNH

A

Eculizumag (inactivates C5)

132
Q

Other Rx PNH

A

Folate

Transfusions

133
Q

What is aplastic anemia

A

Pancytopenia of unknown origin

134
Q

Causes of pancytopenia

A 
Radiation
Toxins: Toluene, DDT, Benzene
Drugs: Phenytoin, Carbamazepine, Chloramphenicol, EtOH, Chemo
SLE
PNH
HIV, Hep, CMV, EBV
B12, Folate def
PTU, Methimazole
135
Q

Presentation for Aplastic Anemia

A

Fatigue
Infections
Bleeding

136
Q

Most accurate test for aplastic anemia

A

BM Bx

137
Q

Supportive therapy for aplastic anemia

A

Transfusions
ABX
Platelets

138
Q

Rx for true aplastic anemia

A

Allogenic BM transplant

139
Q

Rx for true aplastic anemia >50 or unmatched

A 
Antithymocyte globulin (ATG)
Cyclosporine or Tacrolimus (inhibit T cells)
140
Q

Suspected mechanism of aplastic anemia

A

Autoimmune destruction of marrow by T cells

141
Q

What is polycythemia vera

A

Unrestricted proliferation of all 3 cell lines but mainly RBCs

142
Q

Mutation in PV

A

JAK2

143
Q

Sx in PV

A

Hyperviscosity

  • HA, blurred vision, tinnitus
  • HTN
  • Fatigue
  • Splenomegaly
  • Bleeding
  • Thrombosis
144
Q

Lab values in PV

A 
HCT > 60
Red cell mass elevated
O2 NL
Low EPO 
B12 elevated
Low Fe 
Low MCV
145
Q

Most accurate test for PV

A

JAK2 mutation

146
Q

What can PV convert to

A

AML

147
Q

Compare PV to RCC

A

RCC has elevated EPO

148
Q

Rx PV

A

Phlebotomy and aspirin
Hydroxyurea
Allopurinol and Rasburicase
Antihistamines

149
Q

What is essential thrombocytosis

A

Platelets > 1 million causing thrombosis and bleeding

150
Q

What must be excluded in a dx of essential thrombocytosis

A

Stress induced thrombocytosis

  • Cancer
  • Infection
  • Fe def
151
Q

50% pts with essential thrombocytosis have what mutation

A

JAK2

152
Q

When is Rx not necessary in essential thrombocytosis

A
153
Q

What is erythromelalgia

A

Painful red hands in ET

154
Q

Best initial therapy ET

A

Hydroxyurea

155
Q

Rx ET w/ RBC suppression from hydroxyurea

A

Anagrelide

156
Q

Rx erythromelalgia

A

Aspirin

157
Q

What is myelofibrosis

A

Pancytopenia in older pt with BM fibrosis

158
Q

Blood smear features in myelofibrosis

A

Teardrop cells

159
Q

Rx myelofibrosis

A

Thalidomide and Lenalidomide (TNF-inhibitors)

160
Q

Allogenic BM transplant in myelofibrosis

A
161
Q

Sx suggesting acute leukemia

A

Pancytopenia
- Fatigue, infection, bleeding
Hx MDS

162
Q

Translocation in AML M3

A

15, 17

163
Q

Association with M3 AML

A

DIC

164
Q

Best initial test in AML

A

Blood smear showing blasts

165
Q

Most accurate test for AML

A

Flow cytometry

166
Q

What is characteristic of AML

A 
Myeloperoxidase (MPO)
Auer rods (M3)
167
Q

Who gets acute leukemia in general

A

AML - Adults

ALL - Children

168
Q

Mortality in acute leukemia w/o chemo

A

6-12mos

169
Q

BMT mortality in acute leukemia

A

Matched + related = 20%

Matched + unrelated = 30%

170
Q

Initial therapy with AML/ALL

A

Chemo to remove blasts from peripheral smear

171
Q

Rx acute leukemia if prognosis is poor after chemo

A

BMT

172
Q

Best indicator of prognosis in acute leukemia

A

Cytogenetics

173
Q

What is added to chemo for M3

A

All-trans retinoic acid (induces myelocyte maturity)

174
Q

What is added to ALL chemo

A

Intrathecal MTX

175
Q

Chemo drugs in acute leukemia

A

Doxorubicin
VIncristine
Prednisone

176
Q

Function of Rasburicase

A

Prevents tumor lysis related to uric acid rise

177
Q

Differentiate CML from Reactive leukocytosis

A

↑ LAP Reactive

↓ LAP CML

178
Q

Common features of CML

A

High WBC w/ >90% neutrophils
Pruritis after hot baths/showers
Splenomegaly (early satiety, ABD fullness, LUQ pain)
B Sx

179
Q

Most accurate test for CML

A

BCR-ABL on PCR/FISH

180
Q

Best initial therapy CML

A

Tyrosine kinase inhibitors (Gleevec) 90% improvement

181
Q

Only cure for CML

A

BMT

182
Q

Rate of transformation of CML to AML

A

Blast crisis

20% of pts per year

183
Q

Most important step in leukostasis reaction

A

Leukopheresis

  • Remove excess WBCs
  • Hydroxyurea not as fast
184
Q

Characteristic abnormality in MDS

A

5q deletion

185
Q

Who gets MDS

A

> 60 w/ pancytopenia despite hypercellular BM

186
Q

What can MDS conver to

A

AML

Most die of complications of infection or bleeding first

187
Q

Presentation of MDS

A 
ASx pancytopenia on CBC
Fatigue + Wt loss
Infection
Bleeding
Sometimes Splenomegaly
188
Q

Dx tests in MDS

A

CBC anemia w/ ↑MCV, nucleated RBCs, few blasts
Hypercellular marrow
Ringed sideroblasts w/ prussian blue

189
Q

What determines severity of MDS

A

of blasts

190
Q

Most distinct lab abnormality in MDS

A

Pelger-Huet cells

191
Q

Rx MDS

A

Transfusion
EPO (20% response)
Lenalidomide (5q deletion pts)
Azacitidine reduces transfusion dependence but not survival

192
Q

What is CLL

A

Abnormal functioning normal appearing B cells

193
Q

MC Sx in CLL

A

Fatigue

194
Q

Other Sx in CLL

A

Lymphadenoma (80%)
Spleen/Liver enlargement (50%)
Infection

195
Q

What is the Richter phenomenon

A

Conversion of CLL to high grade lymphoma (5% pts)

196
Q

Dx tests in CLL

A

WBC > 20,000 w/ 80-98% lymphocytes
50% pts are hypergammaglobulinemic
Anemia and thrombocytopenia
Smudge cells

197
Q

Stages of chronic leukemia

A 
Stage 0 - ↑WBCs (CLL only)
Stage 1 - +nodes
Stage 2 - Spleen
Stage 3 - Anemia
Stage 4 - ↓platelets
198
Q

Chronic leukemia prognosis by stage w/o rx

A 
0-1 = 10-12yrs
2 = 5 years
3-4 = 1-2yrs
199
Q

Rx CLL stage 2-4

A 
Fludarabine
Refractory - cyclophosphamide
Mild - Chlorambucil
Severe - IVIG
Autoimmune thrombocytopenia or hemolysis - Prednisone
200
Q

Best initial therapy CLL

A

Fludarabine + Rituximab (advanced or sx)

201
Q

Who gets hairy cell leukemia

A

Middle aged men

202
Q

Presentation of hairy cell leukemia

A

Pancytopenia
Massive splenomegaly
Monocytopenia
Dry tap

203
Q

Best initial testof hairy cell leukemia

A

Smear showing hairy cells (B-cells with filamenous projections)

204
Q

Most accurate test for hairy cell leukemia

A

Immunotyping by Flow cytometry

205
Q

Rx Hairy cell leukemia

A

Cladribine or Pentostatin

206
Q

Best initial test for any lymphoma

A

Excisional Bx

207
Q

Presentation of NHL

A 
Painless lymphadenopathy (firm, rubbery, NT)
B Sx
208
Q

CBC in NHL

A

NL

209
Q

Determine severity of NHL

A

High LDH = more severe

210
Q

Staging procedures of NHL

A

CT chest, ABD, pelvis

BM Bx

211
Q

Staging Lymphoma

A

1 - Lymph node group (95% remission)
2 - 2 or more groups on same side of diaphragm
3 - Both sides of diaphragm
4 - Widespread

212
Q

Rx Lymphoma

A

1,2 - Local radiation and chemo

3-4 +/- B Sx - CHOP and rituximab (anti CD20)

213
Q

CHOP

A

Cyclophosphamide
Adriamycin (doxorubicin or hydroxydaunorubicin
Vincristine (Oncovin)
Prednisone

214
Q

MALT associated with

A

H. pylori

215
Q

Rx MALT

A

Clarithromycin and amoxicillin

216
Q

Hodgkin’s Disease specific cells

A

Reed-Sternberg

217
Q

Which lymphoma presents earlier

A

HD - stage 1-2

NHL - stage 3-4

218
Q

Best prognosis in HD

A

Lymphocyte predominant

219
Q

Worst prognosis in HD

A

Lymphocyte depleted

220
Q

Worst prognosis in NHL

A

Burkitt and immunoblastic

221
Q

Rx stage 1-2 HD

A

Local radiation and chemo

222
Q

Rx stage 3-4 HD

A

Adriamycin (doxorubicin)
Bleomycin
Vincristine
Dacarbazine

223
Q

Complications of radiation in HD

A

Breast, Thyroid, Lung tumors

Premature CAD

224
Q

Complications of chemo in HD

A

Acute leukemia, MDS, NHL 1% per year

225
Q

Doxorubicin AE

A

Cardiotoxicity

226
Q

Vincristine AE

A

Neuropathy

227
Q

Bleomycin AE

A

Pulm fibrosis

228
Q

Cyclophosphamide AE

A

Hemorrhagic cystitis

229
Q

Cisplatin AE

A

Renal/Ototoxicity

230
Q

What is myeloma

A

Abnormal proliferation of plasma cells which make IgG and A that don’t fight infection and clog up kidneys

231
Q

MC presentation of myeloma

A

Bone pain 2/2 hypercalcemia and lytic bone lesions

232
Q

What causes lytic bone lesions on myeloma

A

OAF

233
Q

What causes hyperuricemia in myeloma

A

Increased cell turnover of plasma cells

234
Q

What causes anemia in myeloma

A

Infiltration of BM by plasma cells

235
Q

What causes renal failure in myeloma

A

Accumulation of IGs and Bence-Jones
Hypercalcemia
Hyperuricemia

236
Q

First dx test for myeloma

A

XR showing lytic lesions

237
Q

MCC death in myeloma

A

Renal failure and infection

238
Q

Serum protein electrophoresis in Myeloma

A

IgG (60%) or IgA (25%) spike

239
Q

Lab values in myeloma

A 
15% have bence jones protein only
Hypercalcemia
Decreased anion gap
Beta 2 microglobulin
Rouleaux
Elevated BUN/Cr
Elevated total protein and NL albumin
240
Q

Best dx feature of myleoma

A

> 10% plasma cells on BM Bx

241
Q

Why is radionuclide (technetium) scan normal in myeloma

A

Lytic lesions don’t pick up the isotope

242
Q

What detects bence-jones

A

Urine immunoelectrophoresis

243
Q

Best initial therapy for myeloma

A

Dexamethasone with lenalidomide, bortezomib or both

244
Q

Who gets Melphalan in myeloma

A

Older, fragile pts

245
Q

Most effective therapy

A

Autologous BMT w/ stem cell support after induction with chemo (lenalidomide and steroids)

246
Q

SPEP in MGUS

A

IgG or IgA spikes

247
Q

What percentage of MGUS converts to Myeloma

A

1% per year

248
Q

What is Waldenstrom Macroglobulinemia

A

Overproduction of IgM from malignant B cells leading to hyperviscosity

249
Q

Presentation of Waldenstrom

A 
Lethargy
Blurry vision and vertigo
Engorged vessels in the eye
Mucosal bleeding
Raynaud
Anemia
250
Q

Best initial therapy for Waldenstrom

A

Plasmapheresis

251
Q

Long-term rx Waldenstrom

A

Rituximab or Prednisone cyclophosphamide

252
Q

Decrease production of B cells

A

Bortezomib

Lenalidomide

253
Q

Features of platelet bleeding

A

Superficial

Epsitaxis, gingival, petechiae, purpura, mucosal

254
Q

Features of factor bleeding

A

Deep

Joints and Muscles

255
Q

Which organs can have both platelet or factor bleeding

A

Brain

GI

256
Q

What to look for in ITP

A

Isolated thrombocytopenia

Normal-sized spleen

257
Q

Best initial therapy for ITP

A

Prednisone

258
Q

Dx tests for ITP

A

Antiplatelet Abs
U/S or CT for spleen
Megakaryocytes are elevated

259
Q

Rx for no bleeding, plts >30,000 ITP

A

Nothing

260
Q

Rx for mild bleeding, plts

A

Steroids

261
Q

Rx for severe bleeding, plts

A

IVIG, Anti-Rho

262
Q

Rx recurrent episodes, steroid dependent ITP

A

Splenectomy

263
Q

Rx splenectomy, steroid not effective ITP

A

Romiplostim

Eltrobopag, Rituximag, Azathioprine, Cyclosporine, Mycophenolate

264
Q

Fastest way to increase platelet count in ITP

A

IVIG

265
Q

What vaccines to give before splenectomy

A

N. meningitidis
H. influenzae
Pneumococcus

266
Q

Most common inherited bleeding disorder

A

Von Willebrand Disease (AD)

267
Q

What to look for in VW disease

A

Platelet bleeding with NL count

268
Q

What worsens VWD

A

Aspirin

269
Q

Dx tests for VWD

A

Decreased VWF
Ristocetin cofactor assay shows VWF dysfunction
Factor VIII activity
Bleeding time (NEVER done)

270
Q

Best initial therapy for VWD

A

DDAVP

Factor VIII replacement or VWF concentrate if it doesn’t work

271
Q

What to look for in suspected hemophilia

A

Delayed joint/muscle bleeding in male child

272
Q

Lab values in hemophilia

A

NL PT
Prolongued PTT
Mixing studies correct PTT

273
Q

Most accurate test for hemophilia

A

Factor VIII or IX assay

274
Q

Rx mild hemophilia

A

DDAVP

275
Q

Rx Severe bleeding in hemophilia

A

Factor replacement

276
Q

Is there an increase in bleeding in Factor XI deficiency

A

No - only in trauma/surgery

277
Q

Stop bleeding in factor XI deficiency

A

FFP

278
Q

Features of Factor XII deficiency

A

Elevated PTT
No bleeding
NL platelets
No therapy

279
Q

Definite risks of DIC

A 
Sepsis
Burns
Abruptio placentae
Amniotic fluid embolus
Snake bites
Trauma
Cancer
280
Q

Dx tests in DIC

A

Elevated PT and PTT
Low platelets
Elevated D-dimer and fibrin split products
Decreased fibrinogen

281
Q

Rx DIC

A

Platelets

282
Q

MCC thrombophilia

A

Factor V Leiden

283
Q

Rx Factor V Leiden

A

Warfarin till INR 2-3 for 6mos

284
Q

Causes of HIT

A

Unfractionated > LMWH

285
Q

When does HIT present

A

5-10 days after starting heparin

286
Q

How does HIT present

A

Platelets drop by 30%

Venous clots > arterial clots

287
Q

Dx tests for HIT

A

ELISA for platelet factor 4 abs or serotonin release assay

288
Q

Rx HIT

A

Stop all heparin products
Direct thrombin inhibitors - Agatroban, Lepirudin, Bivalrudin
Warfarin after inhibitors

289
Q

2 main syndromes in Antiphospholipid syndromes (APL)

A

Lupus anticoagulant

Anticardiolipin Ab

290
Q

Anticardiolipin association

A

Spontaneous abortions

291
Q

Only abnormality in APL

A

PTT

292
Q

Best initial test in APL

A

Mixing study

293
Q

Most accurate test for APL

A

Russell viper venom test for lupus anticoagulant

294
Q

Rx APL

A

Heparin and warfarin

May be lifeling

295
Q

What makes ITP worse

A

Aspirin

Step 2 CK (21 decks)

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