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Didactic Year > Heme-Onc > Flashcards

Heme-Onc Flashcards

1
Q

(ITP) Immune Thrombocytopenia Purpura

A

PLT <100,000 normal WBC and Hgb
Isolated thrombocytopenia on CBC

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2
Q

Acute ITP

A

Children preceded by viral infection (1-3 weeks)
Spontaneously resolves
Usually petechial rash

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3
Q

Chronic ITP

A

Lasts >12 months
Females
20-40 y/o
Maybe associated with states of altered immunity

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4
Q

PLT <30,000 with 1 other risk factor in ITP (children)

A

Wet purpura
Epistaxis >5 min
Hematuria or hematochezia
Menorrhagia
On anticoagulation
Known underlying bleeding d/o (vWD)
Limited medical care; follow up cannot be assured

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5
Q

First line treatment for ITP

A

Steroids or IVIG

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6
Q

Relapse or persistent ITP - 2nd line tx

A

Rituximab
Or
Eltrombopag

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7
Q

ITP tx in the setting of critical bleeding

A

Triple therapy= PLT transfusion, Steroids, IVIG

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8
Q

Response to treatment for ITP

A

PLT >30,000
and
PLT count double from the time of Dx

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9
Q

Thrombotic thrombocytopenic Purpura (TTP)

A

ADAMTS13 enzyme defect/deficiency
Females 2:1 and AA
Thrombocytopenia
Microthrombi occlusion
Hemolytic anemia

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10
Q

Hallmark clinical Pentad (FATRN) of TTP

A
  1. Fever
  2. Microangiopathic Hemolytic Anemia
    - Shistocytes in the peripheral blood smear *
  3. Thrombocytopenia *
  4. Renal disease- mild~moderate
  5. Neurological abnormalities
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11
Q

TTP Dx

A

hgb <10, PLT <30,000
Schistocytes
DO NOT WAIT for ADAMTS13 activity level/antibody testing

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12
Q

TTP first line tx

A

(Plasmapheresis) Plasma exchange

Steroids given if not available or in combo

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13
Q

Tx once ADAMTS13 confirmed and activity level <10%

A

Rituximab

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14
Q

Caplacizumab

A

Used as additional therapy if neurological sxs in TTP or refractory

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15
Q

Classic Hemolytic Uremic Syndrome- HUS triad

A

Microangiopathic hemolytic anemia
Acute kidney injury*
Thrombocytopenia

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16
Q

Etiology of HUS

A

E. coli 0157:H7 classic exotoxin (“Shiga toxin”)
(undercooked beef,water contamination from feces,unpasteurized milk)
PRODROMAL gastroenteritis

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17
Q

HUS patients

A

Mostly children or immunocompromised adults

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18
Q

HUS Workup

A

Thrombocytopenia
Hemolytic normocytic anemia
Schistocytes
ELEVATED BUN & Cr on bmp
Stool cx shows E. Coli or Shigella
Normal ADAMTS13

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19
Q

HUS No Nos

A

No antibiotics No anti motility agents

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20
Q

Etiology of DIC

A

Complication due to another pathological condition=
Sepsis/trauma
or
Malignancy (APL)
or OB complication Ex. Abruptio placenta

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21
Q

Disseminated Intravascular Coagulation (DIC)

A

Consumption of clotting factors and platelets
Oozing blood from every orifice (IV lines etc.)

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22
Q

DIC labs

A

PT and PTT are elevated
D-Dimer is elevated
Fribrinogen is low
Bleeding time is prolonged
(also thrombocytopenia, Schistocytes)

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23
Q

DIC Management

A

Keep Fibrinogen level >100
Cryoprecipitate
Correcting PT and PTT with
FFP (fresh frozen plasma)
Keep PLT >50k in serious bleeding with transfusions

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24
Q

Heparin Induced Thrombocytopenia (HIT) type 2

A

PF4 on Heparin recognized as foreign= HIT antibodies attaches to Fc receptor and activates Platelet causing clot formation
TYPICALLY within 5-10 days from Heparin exposure

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25
Q

HIT labs

A

PLT drop by 50% after heparin exposure

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26
Q

HIT Presentation

A

Necrotic skin lesions at site of Heparin injection or IV site

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27
Q

4 Ts score for HIT

A

Thrombocytopenia
Timing
Thrombosis
oTher causes for the thrombocytopenia ruled out

Score of 4-8 tx!

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28
Q

Confirmatory test for HIT

A

Serotonin release assay (measures platelet activation)

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29
Q

Anticoagulation after HIT Dx

A

At least 4 weeks on a direct thrombin inhibitor or DOAC

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30
Q

Primary Hemostasis

A

Constriction of vessel
Formation of platelet plug (vW)
Issue=
- skin/mucosa bleeding
Prolonged bleeding time or PFA (PLT Functional Assay)
Normal PLT count

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31
Q

Secondary Hemostasis

A

Coagulation cascade of clotting factors
Issue=
- deeper bleeding
Prolonged PT or PTT

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32
Q

Tertiary Hemostasis

A

Fibrinolysis
Issue=
Hypercoagulation

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33
Q

Extrinsic pathway

A

PT
Factor VII (7)

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34
Q

Intrinsic pathway

A

aPTT
Factor XII, XI, IX, VIII
(12,11,9,8)

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35
Q

Vitamin K deficiency

A

Factor VII (7) depleted first
Seen in elderly pts, very sick, or neonates
Need fat to absorb

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36
Q

Hemophilia A

A

Factor 8 deficiency
Prolonged PTT

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37
Q

Hemophilia B

A

Factor 9 deficiency
Prolonged PTT

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38
Q

Von Willebrand protects what factor?

A

Factor VIII (8)
(Von Wille disease can lead to prolonged PTT and deeper bleeding)

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39
Q

Mixing studies

A

Mix pt blood with control plasma
Distinguishes factor deficiency from factor inhibitors
PT/PTT will correct if missing a factor
PT/PTT will NOT correct if inhibitor

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40
Q

Ristocetin

A

Activity is decreased in vWD

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41
Q

Von Willebrand disease etiology

A

Inherited autosomal dominant disorder
Most common inherited bleeding disorder
Most have mild form
3 types
Dx after prolonged bleeding after dental procedure or female heavy periods

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42
Q

Type 1 vW

A

Most common
Sxs mild
Low quantity

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43
Q

Type 2 vW

A

Quality issue
Sxs include epistaxis for more than 10 min

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44
Q

Type 3 vW

A

Little or no vWf
Not protecting factor 8 ~ factor bleeding
Prophylaxis with vWF infusions

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45
Q

Desmopressin (DDAVP)

A

Causes the release of vWF and factor VIII*
Tx of choice for type 1

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46
Q

Patient education in vWD

A

AVOID Aspirin/NSAID

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47
Q

Actively bleeding in vWD

A

Tx with vWF concentrate or Factor 8(contains vWF)

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48
Q

“Christmas disease”

A

Hemophilia B

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49
Q

Etiology of Hemophilia

A

X-linked recessive disorder
Seen more in males
Hallmark- Hemarthrosis- bleeding into joints
Post circumcision bleeding may be first indication

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50
Q

Emicizumab

A

Only indicated for Hemophilia A (bypasses factor 8)

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51
Q

Hemophilia Patient Education

A

No high impact sports

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52
Q

Vit K dependent factors

A

X,IX,VII,II
10,9,7,2

Protein C & S (natural anticoagulants)

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53
Q

Vit K deficiency etiology

A

Poor diet,fat malabsorption
WARFARIN use
Chronic liver Dz
BROAD SPECTRUM antibiotics use

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54
Q

Vit K classic patient

A

Neonates born outside of hospital
Or
Postop poor po intake and on antibiotics

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55
Q

Vit K deficiency clinical feature

A

Soft tissue bleeding (hematomas)

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56
Q

Acute hemorrhage in Vit k deficiency

A

Give pt fresh frozen plasma (contains all clotting factors) + Vit K

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57
Q

Protein C & S inhibits

A

Factor V and VIII

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58
Q

Antithrombin inhibits

A

Thrombin(IIa)
and
Factor X

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59
Q

Increased risk for inherited coagulation disorder

A

First degree relative with VTE prior to age 45

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60
Q

Factor V Leiden

A

Most common inherited hypercoaguable disorder
-Caucasians
Autosomal dominant point mutation
Resistance to activated Protein C
-DVT/PE/Fetal loss

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61
Q

Activated Protein C Resistance Assay

A

In pt with factor V Leiden
PTT stays the same

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62
Q

Prothrombin Gene Mutation G20210A

A

2nd most common
Autosomal dominant point mutation
-Caucasians
Inc amt of prothrombin
DNA Analysis
-DVT/miscarriage/Cerebral Vein thrombosis

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63
Q

Protein C & S deficiency

A

Neonatal Purpura Fulminans (rare life threatening)

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64
Q

Warfarin induced skin necrosis

A

Protein C deficiency following tx with Warfarin
transient Hypercoaguability

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65
Q

Tx for Warfarin induced skin necrosis

A

Immediately discontinue warfarin
Give IV Vit K
Start heparin
Give protein C concentrate

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66
Q

Antithrombin deficiency

A

Acquired form more common (ex. Nephrotic syndrome)
Insensitivity to heparin
DVT/PE/Stroke

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67
Q

Antithrombin-heparin cofactor assay

A

Positive test when Antithrombin activity <80% of the normal range and
PTT doesn’t prolong

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68
Q

Inherited thrombophilia prophylaxis

A

-pregnancy with LMWH
-postoperatively

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69
Q

Antiphospholipid antibody syndrome

A

Young females
Acquired
Lupus
-Recurrent miscarriages
MILD Thrombocytopenia

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70
Q

Skin rash in Antiphospho (APS)

A

Livedo reticularis
(Reticular LACY violaceous rash)

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71
Q

Endocarditis associated with APS

A

Libman-Sacks endocarditis
Sterile
Commonly affects the mitral valve

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72
Q

Russel Viper Venom test

A

Prolonged PTT in vitro

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73
Q

Hodgkin Lymphoma

A

Bimodal age of onset
Males
Less common than NHL
B-cell origin
EBV

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74
Q

Classic HL

A

Reed sternberg cell
Nodular sclerosis is most common

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75
Q

Owl eyes

A

Reed Sternberg cell

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76
Q

Reason to do excisional biopsy in HL

A

Ex. May miss reed sternberg cell in Lymphocyte predominance

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77
Q

S/s of HL

A

Painless lymphadenopathy above diaphragm
1. Cervical chains
2. Mediastinal
Contiguous manner
Constitutional sxs
Pruritis
Painful adenopathy after ETOH ingestion

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78
Q

Pel-Ebstein fever in HL

A

Cyclic fever

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79
Q

Staging difference in HL

A

CT neck

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80
Q

Tx for HL

A

ABVD
(adriamycin, bleomycin, Vinblastine,Dacarbazine)

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81
Q

Adriamycin

A

Anthracycline (Cardiotoxicity)

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82
Q

Non-Hodgkin Lymphoma (NHL)

A

B-cell more common
1. indolent (low grade)
2. aggressive (high grade)
Pesticides like Round Up
No “B” sxs in indolent type
Adenopathy is NOT in contiguous pattern
Extranodal involvement

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83
Q

Indolent NHL

A

Follicular lymphoma (most common)
Marginal Zone lymphoma
Waldenstrom

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84
Q

Aggressive NHL

A

Diffuse Large B-cell
Burkitt Lymphoma

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85
Q

EBV

A

Burkitt lymphoma
HL

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86
Q

Gastric MALToma

A

H. Pylori Marginal Zone lymphoma

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87
Q

Differences in staging in NHL

A

Bone marrow biopsy
and
Lumbar puncture

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88
Q

Burkitt Lymphoma

A

T(8,14)
Can cross BBB
Children and young adults
In Africa- facial and jaw extranodal
US- Abdominal mass
“Starry sky” histology - tingible-body macrophages

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89
Q

Tx for Burkitt

A

HyperCVAD regimen
or
R-EPOCH

90
Q

Diffuse Large B-Cell (DLBC)

A

Most common NHL
extranodal masses- mediastinum

91
Q

Tx for DLBC

A

R-CHOP
Rituximab
Cyclophos
H (doxorubicin)
O (vincristine)
Prednisone

92
Q

Follicular Lymphoma

A

T(14;18)
Can transform DLBC
Early stages- radiation alone

93
Q

Tx for later stages of Follicular lymphoma

A

Rituximab
Or
With Bendamustine
or CVP (Cyclophos,Vincris,Prednisone)

94
Q

Marginal Zone Lymphoma

A
  1. Extranodal marginal zone lymphoma
95
Q

Extranodal marginal zone lymphoma (AKA MALT)

A

In organs that contain mucosa
1. GI tract
Thyroid, eyes, and lungs

96
Q

MALT tx fail H. Pylori

A

Radiation
Rituximab or
Chemotherapy

97
Q

Waldenstrom Macroglobulinemia
(AKA lymphoplasmacytic lymphoma)

A

Excessive IgM
Risk factor- MGUS

98
Q

S/s of walden

A

Raynaud’s phenomenon (Cold autoimmune hemolytic anemia-IgM)
PLT related bleeding (gums,nose)
Viscosity - requires plasmapheresis
Peripheral Neuropathy
Hepatosplenomegaly/Lymphadenopathy

99
Q

OVA mnemonic for Walden

A

Organ infiltration
Viscosity
Anemia

100
Q

(SPEP) Serum protein Electrophoresis

A

Monoclonal spike in gamma region result then need to do immunofixation to see IgM in Walden or IgG/IgA in MM

101
Q

Rouleaux

A

Stacked coin appearance on peripheral smear seen in both Waldenstrom and MM

102
Q

Tx for Walden

A

Sxs=
Rituximab or Ibrutinib single agent or with chemo

103
Q

Multiple Myeloma
(Plasma cell myeloma)

A

IgG- most common
AA
Agent orange
MGUS

104
Q

MM B.R.E.A.K clinical features

A

Bone pain
Recurrent infections
Elevated calcium
Anemia
Kidney damage - Bence Jones protein

Most because of osteoclast activity

105
Q

Lytic lesions

A

MM
“Punched out”
Vertebral fractures - back pain
NOT ON BONE SCAN

106
Q

UPEP (urine protein electrophoresis)

A

M-spike in urine
Bence Jones Protein

107
Q

Tx for MM

A

2.Auto SCT
1.Combo chemo (VRD or RVD) =
Velcade(Borte), Revlimid*, Dexamethasone
Sxs tx- Bisphosphonates
Plasmacytoma- Radiation

108
Q

MGUS

A

Lifelong monitoring Q6months
-SPEP/UPEP
-CBC
-BMP

109
Q

Chronic leukemia

A

Insidious onset
Less aggressive
Mature forms

110
Q

Acute Leukemia

A

Rapid onset
Aggressive disease
Immature cells (BLASTS forms)

111
Q

AML

A

Adult pop
Caucasians
Males* MDS risk
Down syndrome
“Chimney sweeps”

112
Q

Sxs of AML

A

Anemia
Neutropenia
Thrombocytopenia
Bone pain

113
Q

M3 subtype of AML

A

APL
DIC

114
Q

First clinical presentation of AML

A

Oncological emergencies-
Neutropenic fever or
Leukostasis
or
Tumor Lysis syndrome

115
Q

Neutropenic fever

A

ANC <1500
101 F or 100.4 over an hour

116
Q

Tx for Neutropenia fever

A

Panculture
Broad spectrum antibiotics within 60 min (cover Pseudomonas)

117
Q

Tumor Lysis Syndrome P.U.C.K

A

Hyper phosphatemia
Hyper uricemia
Hypo calcemia
Hyper kalemia

118
Q

Signs and Sxs of tumor lysis

A

Decreased urine output
Muscle cramps
N/v
Lethargy
Arrhythmias
AKI
Seizure
100,000 or higher WBC, all Blast cells

119
Q

Tumor lysis tx

A

Aggressive IVF
Allopurinal or rasburicase (uric acid >14)
Phoslo
Calcium gluconate

120
Q

Leukostasis

A

Dyspnea!
Altered mental stasis!
HA
Retinal thrombus
Priapism
Dec urine output

121
Q

Leukostasis tx

A

Leukapheresis first line

122
Q

AML physical findings

A

Gingival Hyperplasia
Leukemia cutis (more in relapsed Dz)
Granulocytic sarcoma (spleen more common and in subtype M5)

123
Q

Peripheral blood smear in AML/APL

A

Auer rods

124
Q

Myeloperoxidase

A

BLASTS stain positive

125
Q

Prior to initiation of AML tx

A

ECHO - anthracycline
Central line
CMP

126
Q

Prophylactic regimen for AML

A

Antibiotic- Levaquin
Antiviral- Valtrex
Antifungal- Posaconazole or Fluconazole

127
Q

Induction Chemo for AML

A

7+3
Cytarabine + Anthracycline

128
Q

Consolidation chemo for AML

A

HIDAC
High dose cytarabine

129
Q

Acute Promyelocytic Leukemia (APL)

A

T(15;17)
Younger patients
No SCT
Wet purpura
Fibrinogen less than 125- replace with Cryoprecipitate

130
Q

Tx for APL

A

ATRA (Vit A) plus chemo or arsenic

131
Q

(ALL) Acute Lymphoblastic leukemia

A

Most common type of cancer in children- boys
Down syndrome
Previous malignancy
Poorer prognosis in adults

132
Q

T-cell ALL

A

Teenagers
Thymus enlargement
B-cell is more common

133
Q

ALL s/s

A

Bone pain more common complaint
Headaches- crosses BBB
Infiltration to extramedullary (ex. lymphadenopathy)

134
Q

(TdT) Terminal deoxynucleotidyl transferase

A

Positive stain in immature lymphoblasts

135
Q

Prior to tx in ALL

A

LP

136
Q

Prophylaxis for ALL includes

A

Bactrim or Dapsone for PCP/PJP ~pneumonia

137
Q

CNS prophylaxis in ALL

A

Methotrexate or Cytarabine
Hydrocortisone

138
Q

CML

A

Philadelphia chromosome T(9;22)
Atomic bomb pt

139
Q

S/s of CML

A

Abdominal fullness
Early satiety
Splenomegaly

140
Q

LAP (leukocyte alkaline phosphatase) Score

A

CML low Lap score

141
Q

Tx for CML

A

TKIs first line
Imatinib (gleevac)
Proven cure- Allogenic SCT

142
Q

CLL

A

Most common leukemia of adults
Lymphadenopathy!
B-cell

143
Q

Isolated lymphocytosis

A

> 5000
CLL

144
Q

Warm autoimmune hemolytic anemia

A

Sxs- jaundice, tea colored urine
Spherocytes- fat guy in a little suit
Coombs +
IgG
-CLL

145
Q

Smudge cells

A

Incompetent B cells
CLL!!!!

146
Q

CD19,20,23,5

A

CLL

147
Q

Venetoclax

A

CLL tx

148
Q

Polycythemia Vera

A

Most common MPD
Predominance in RBC’s
HCT >49% in M and >48% in F
JAK2 mutation (not driven by EPO)

149
Q

PV sxs

A

Retinal vein engorgement
Aquagenic pruritis
Plethora (ruddy appearance of face and palms)
Erythromelalgia -relieved by aspirin
Hyperviscosity sxs incl HA,confusion
Risk for gout

150
Q

PV Dx

A

CBC
-WBC shows elevated eosinophils and basophils
LOW EPO Levels

151
Q

Tx for PV

A

Phlebotomy to HCT <45%
ASA 81 mg

152
Q

What can PV transform to?

A

AML
or
PM

153
Q

Which gender is ET predominant?

A

Females

154
Q

Essential thrombocytosis (ET)

A

PLT >450,000

155
Q

Mutations in ET

A

JAK2
CALR
MPL

156
Q

Miscarriages in 1st trimester

A

ET

157
Q

Myelocytes & Metamyelocytes

A

ET

158
Q

Peripheral smear in ET

A

Giant PLTs

159
Q

BMBX in ET

A

Increased megakaryocytes

160
Q

Primary Myelofibrosis (PMF)

A

Chronic myeloproliferative disorder
Extramedullary hematopoiesis
Enlarged spleen!!!!!!!!!!! CLASSIC
Anemic first

161
Q

Peripheral Smear triad in PMF

A
  1. Leukoerythroblastosis
    -nucleated RBC etc
  2. Teardrop-shaped RBCs
  3. Giant abnormal PLT w/o granules
162
Q

BMBX in PMF

A

Dry tap

163
Q

What drugs can decrease spleen size in PMF

A

Hydroxyurea
or
Ruxolitnib (Jakafi)

164
Q

Myelodysplastic syndrome (MDS)

A

Dysplastic immature cells-nonfunctional
Early apoptosis

165
Q

MDS

A

Increased incident to transform to AML
Exposure to heavy metals -lead

166
Q

Primary MDS

A

Idiopathic
Most common

167
Q

Secondary MDS

A

Chemo/radiation exposure

168
Q

What anemia is found in MDS?

A

Macrocytic anemia

169
Q

Peripheral blood smear in MDS

A

Dysplastic RBCs
Hyposegmented neutrophils

170
Q

ANC in MDS

A

ANC <1800

171
Q

Low risk MDS med management

A

Azacitidine or Decitabine

172
Q

Hgb F

A

2 alpha 2 gamma
Highest in utero

173
Q

Hgb A

A

2 alpha 2 beta
Highest 6 months of age

174
Q

High retic

A

Hemolysis or bleeding

175
Q

Low retic

A

Bone marrow issue or
Missing ingredient

176
Q

What medications can raise WBC?

A

Steroids, Epinephrine

177
Q

What is the most common cause of Leukopenia?

A

Neutropenia
Severe infection risk ANC <500

178
Q

Anisocytosis

A

Variation in erythrocyte size

179
Q

Pagophagia, Pica

A

Iron deficiency anemia

180
Q

Hemolytic anemias

A

Jaundice
Icterus
Pruritis
Hemoglobinuria

181
Q

COOMBs test

A

Autoimmune testing

182
Q

What is the most common anemia worldwide?

A

Iron deficiency anemia- dietary cause
(Blood loss cause in US)

183
Q

Fe deficiency anemia pathognomonic

A

Ferritin less than 20

184
Q

Hgb A2

A

2 alpha and 2 delta

185
Q

Thalassemia

A

Microcytic anemia with normal iron studies
-target cells
Hemolysis labs

186
Q

Hemolysis labs

A

Haptoglobin -taxi (dec)
LDH (inc)
Indirect bili (inc)
High retic

187
Q

Alpha thalassemia

A

Southeast Asia and Africa
2 genes defective=
Look at iron studies or will miss dx

188
Q

Hgb H disease

A

Alpha thalassemia intermedia=
3 genes defective
Splenomegaly
Boney deformities= frontal bossing
Hgb Electrophoresis

189
Q

Hydrops Fetalis

A

Alpha thalassemia major
4 genes defective
Still borns

190
Q

Beta Thalassemia

A

2 beta genes on Chromosome 11
Mediterranean origin

191
Q

Beta thalassemia minor

A

1 gene defective
Increased Hgb A2

192
Q

Beta thalassemia Major: Cooley’s anemia

A

Hgb F predominant
Transfusion dependent
So need Chelation therapy (Deferasirox)

193
Q

Sideroblastic anemia

A

More commonly acquired-
Chronic ETOH
Lead
Tx with isoniazid for TB
Vitamin B6 deficiency
Sxs- irritability in children, cog impairment

194
Q

Sideroblastic anemia work up

A

MCV <80
Basophilic stippling- Lead poisoning
BMBX confirms with “ringed sideroblasts”
Lead Lines in children

195
Q

Sideroblastic anemia tx

A

Supplementation with Vit B6
Lead poisoning- Succimer

196
Q

Lead level 70 or higher (severe)

A

Succimer plus IV Calcium disodium EDTA

197
Q

Vitamin B12 (cobalamin) deficiency

A

Strict vegan diet at risk
Pernicious anemia autoimmune dz is main cause
“Stocking and glove paresthesia”
Macro-Ovalocyte and Hypersegmented =Megablastic anemia

198
Q

Folate deficiency anemia

A

Alcoholic or Anorexic pts
-methotrexate
Chronic hemolytic anemia

199
Q

Anemia of chronic disease

A

EPO is suppressed
Hepcidin increases (iron regulator)
TIBC decrease and Ferritin (iron stores) high
Serum iron is decreased
ESR and CRP is elevated

200
Q

Aplastic anemia

A

Pancytopenia
80% acquired - idiopathic
Autoimmune suppression/destruction by T-cells
Hypoplastic bone marrow

201
Q

BMBX in Aplastic anemia

A

Hypocellular marrow

202
Q

Severe cases of Aplastic anemia (Adults >40,no BMT donor) tx

A

ATG (antithymocyte globulin) + Cyclosporin [Both target T cells] and a bone marrow stimulating agent Eltrombopag

203
Q

Hereditary Spherocytosis

A

Hyperchromic anemia- MCHC elevated
Pigmented gallstones
Coombs neg

204
Q

Confirmation tests for Hereditary Spherocytosis

A

EMA binding (Eosin-5-maleimide binding test)

205
Q

Tx for Hereditary Spherocytosis

A

Splenectomy

206
Q

G6PD deficiency

A

X-linked, males
African and Mediterranean
Stressors like fava beans
Heinz bodies- Bite cells
Episodic hemolysis

207
Q

Sickle cell Anemia

A

Autosomal recessive disorder that affects beta globin gene

208
Q

Dactylitis

A

Sickle cell

209
Q

Complication of sickle cell

A

Avascular necrosis - femoral head(MC)

210
Q

Risk of nonfunctional spleen (by 6 years of age)

A

Infections from encapsulated bacteria

211
Q

Splenic sequestration crisis

A

Children, low blood pressure etc
Can be fatal within hours
Aggressive transfusion needed

212
Q

Aplastic Crisis

A

Sickle cell pt
Most common etiology is Parvovirus-19 (fifth disease)
Need to be hospitalized to watch H & H

213
Q

Howell Jolly bodies

A

Due to nonfunctional spleen/no spleen
Can be seen by the naked eye

214
Q

Autoimmune Hemolytic Anemia

A

Warm- females, Lupus,Beta-lactams,
Splenomegaly*

Cold- EBV,Mycoplasma PNA

215
Q

Workup for warm Antibody Hemolytic anemia

A

MCHC elevated
+ Coombs test
Cold Agglutinin test- Negative
Blood smear can show Spherocytes

216
Q

Tx in Warm

A

First line Glucocorticoids
2nd line is Rituximab

217
Q

Cold Antibody Hemolytic Anemia

A

Always normocytic
COOMBs positive for compliment only
Confirmatory test is cold agglutinin titer
Avoid cold or use Rituximab

218
Q

(PNH) Paroxysmal Nocturnal Hemoglobinuria

A

Acquired mutation
PIGA gene
Decrease of CD55 and CD59 (confirmatory test of flow cytometry) so increased compliment binding

219
Q

PNH triad

A

Hemolytic anemia
Thrombus in unusual sites
Coca-cola urine (clearer as day progresses)
Sxs- erectile dysfunction and esophageal spasm

220
Q

Tx for PNH

A

Anti-compliment therapy with
Ravulizumab

Didactic Year (24 decks)

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