Heme-Onc Flashcards
1
Q
(ITP) Immune Thrombocytopenia Purpura
A
PLT <100,000 normal WBC and Hgb
Isolated thrombocytopenia on CBC
2
Q
Acute ITP
A
Children preceded by viral infection (1-3 weeks)
Spontaneously resolves
Usually petechial rash
3
Q
Chronic ITP
A
Lasts >12 months
Females
20-40 y/o
Maybe associated with states of altered immunity
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
5
Q
First line treatment for ITP
A
Steroids or IVIG
6
Q
Relapse or persistent ITP - 2nd line tx
A
Rituximab
Or
Eltrombopag
7
Q
ITP tx in the setting of critical bleeding
A
Triple therapy= PLT transfusion, Steroids, IVIG
8
Q
Response to treatment for ITP
A
PLT >30,000
and
PLT count double from the time of Dx
9
Q
Thrombotic thrombocytopenic Purpura (TTP)
A
ADAMTS13 enzyme defect/deficiency
Females 2:1 and AA
Thrombocytopenia
Microthrombi occlusion
Hemolytic anemia
10
Q
Hallmark clinical Pentad (FATRN) of TTP
A
- Fever
- Microangiopathic Hemolytic Anemia
- Shistocytes in the peripheral blood smear * - Thrombocytopenia *
- Renal disease- mild~moderate
- Neurological abnormalities
11
Q
TTP Dx
A
hgb <10, PLT <30,000
Schistocytes
DO NOT WAIT for ADAMTS13 activity level/antibody testing
12
Q
TTP first line tx
A
(Plasmapheresis) Plasma exchange
Steroids given if not available or in combo
13
Q
Tx once ADAMTS13 confirmed and activity level <10%
A
Rituximab
14
Q
Caplacizumab
A
Used as additional therapy if neurological sxs in TTP or refractory
15
Q
Classic Hemolytic Uremic Syndrome- HUS triad
A
Microangiopathic hemolytic anemia
Acute kidney injury*
Thrombocytopenia
16
Q
Etiology of HUS
A
E. coli 0157:H7 classic exotoxin (“Shiga toxin”)
(undercooked beef,water contamination from feces,unpasteurized milk)
PRODROMAL gastroenteritis
17
Q
HUS patients
A
Mostly children or immunocompromised adults
18
Q
HUS Workup
A
Thrombocytopenia
Hemolytic normocytic anemia
Schistocytes
ELEVATED BUN & Cr on bmp
Stool cx shows E. Coli or Shigella
Normal ADAMTS13
19
Q
HUS No Nos
A
No antibiotics No anti motility agents
20
Q
Etiology of DIC
A
Complication due to another pathological condition=
Sepsis/trauma
or
Malignancy (APL)
or OB complication Ex. Abruptio placenta
21
Q
Disseminated Intravascular Coagulation (DIC)
A
Consumption of clotting factors and platelets
Oozing blood from every orifice (IV lines etc.)
22
Q
DIC labs
A
PT and PTT are elevated
D-Dimer is elevated
Fribrinogen is low
Bleeding time is prolonged
(also thrombocytopenia, Schistocytes)
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
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
25
HIT labs
PLT drop by 50% after heparin exposure
26
HIT Presentation
Necrotic skin lesions at site of Heparin injection or IV site
27
4 Ts score for HIT
Thrombocytopenia
Timing
Thrombosis
oTher causes for the thrombocytopenia ruled out
Score of 4-8 tx!
28
Confirmatory test for HIT
Serotonin release assay (measures platelet activation)
29
Anticoagulation after HIT Dx
At least 4 weeks on a direct thrombin inhibitor or DOAC
30
Primary Hemostasis
Constriction of vessel
Formation of platelet plug (vW)
Issue=
- skin/mucosa bleeding
Prolonged bleeding time or PFA (PLT Functional Assay)
Normal PLT count
31
Secondary Hemostasis
Coagulation cascade of clotting factors
Issue=
- deeper bleeding
Prolonged PT or PTT
32
Tertiary Hemostasis
Fibrinolysis
Issue=
Hypercoagulation
33
Extrinsic pathway
PT
Factor VII (7)
34
Intrinsic pathway
aPTT
Factor XII, XI, IX, VIII
(12,11,9,8)
35
Vitamin K deficiency
Factor VII (7) depleted first
Seen in elderly pts, very sick, or neonates
Need fat to absorb
36
Hemophilia A
Factor 8 deficiency
Prolonged PTT
37
Hemophilia B
Factor 9 deficiency
Prolonged PTT
38
Von Willebrand protects what factor?
Factor VIII (8)
(Von Wille disease can lead to prolonged PTT and deeper bleeding)
39
Mixing studies
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
40
Ristocetin
Activity is decreased in vWD
41
Von Willebrand disease etiology
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
42
Type 1 vW
Most common
Sxs mild
Low quantity
43
Type 2 vW
Quality issue
Sxs include epistaxis for more than 10 min
44
Type 3 vW
Little or no vWf
Not protecting factor 8 ~ factor bleeding
Prophylaxis with vWF infusions
45
Desmopressin (DDAVP)
Causes the release of vWF and factor VIII*
Tx of choice for type 1
46
Patient education in vWD
AVOID Aspirin/NSAID
47
Actively bleeding in vWD
Tx with vWF concentrate or Factor 8(contains vWF)
48
“Christmas disease”
Hemophilia B
49
Etiology of Hemophilia
X-linked recessive disorder
Seen more in males
Hallmark- Hemarthrosis- bleeding into joints
Post circumcision bleeding may be first indication
50
Emicizumab
Only indicated for Hemophilia A (bypasses factor 8)
51
Hemophilia Patient Education
No high impact sports
52
Vit K dependent factors
X,IX,VII,II
10,9,7,2
Protein C & S (natural anticoagulants)
53
Vit K deficiency etiology
Poor diet,fat malabsorption
WARFARIN use
Chronic liver Dz
BROAD SPECTRUM antibiotics use
54
Vit K classic patient
Neonates born outside of hospital
Or
Postop poor po intake and on antibiotics
55
Vit K deficiency clinical feature
Soft tissue bleeding (hematomas)
56
Acute hemorrhage in Vit k deficiency
Give pt fresh frozen plasma (contains all clotting factors) + Vit K
57
Protein C & S inhibits
Factor V and VIII
58
Antithrombin inhibits
Thrombin(IIa)
and
Factor X
59
Increased risk for inherited coagulation disorder
First degree relative with VTE prior to age 45
60
Factor V Leiden
Most common inherited hypercoaguable disorder
-Caucasians
Autosomal dominant point mutation
Resistance to activated Protein C
-DVT/PE/Fetal loss
61
Activated Protein C Resistance Assay
In pt with factor V Leiden
PTT stays the same
62
Prothrombin Gene Mutation G20210A
2nd most common
Autosomal dominant point mutation
-Caucasians
Inc amt of prothrombin
DNA Analysis
-DVT/miscarriage/Cerebral Vein thrombosis
63
Protein C & S deficiency
Neonatal Purpura Fulminans (rare life threatening)
64
Warfarin induced skin necrosis
Protein C deficiency following tx with Warfarin
transient Hypercoaguability
65
Tx for Warfarin induced skin necrosis
Immediately discontinue warfarin
Give IV Vit K
Start heparin
Give protein C concentrate
66
Antithrombin deficiency
Acquired form more common (ex. Nephrotic syndrome)
Insensitivity to heparin
DVT/PE/Stroke
67
Antithrombin-heparin cofactor assay
Positive test when Antithrombin activity <80% of the normal range and
PTT doesn’t prolong
68
Inherited thrombophilia prophylaxis
-pregnancy with LMWH
-postoperatively
69
Antiphospholipid antibody syndrome
Young females
Acquired
Lupus
-Recurrent miscarriages
MILD Thrombocytopenia
70
Skin rash in Antiphospho (APS)
Livedo reticularis
(Reticular LACY violaceous rash)
71
Endocarditis associated with APS
Libman-Sacks endocarditis
Sterile
Commonly affects the mitral valve
72
Russel Viper Venom test
Prolonged PTT in vitro
73
Hodgkin Lymphoma
Bimodal age of onset
Males
Less common than NHL
B-cell origin
EBV
74
Classic HL
Reed sternberg cell
Nodular sclerosis is most common
75
Owl eyes
Reed Sternberg cell
76
Reason to do excisional biopsy in HL
Ex. May miss reed sternberg cell in Lymphocyte predominance
77
S/s of HL
Painless lymphadenopathy above diaphragm
1. Cervical chains
2. Mediastinal
Contiguous manner
Constitutional sxs
Pruritis
Painful adenopathy after ETOH ingestion
78
Pel-Ebstein fever in HL
Cyclic fever
79
Staging difference in HL
CT neck
80
Tx for HL
ABVD
(adriamycin, bleomycin, Vinblastine,Dacarbazine)
81
Adriamycin
Anthracycline (Cardiotoxicity)
82
Non-Hodgkin Lymphoma (NHL)
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
83
Indolent NHL
Follicular lymphoma (most common)
Marginal Zone lymphoma
Waldenstrom
84
Aggressive NHL
Diffuse Large B-cell
Burkitt Lymphoma
85
EBV
Burkitt lymphoma
HL
86
Gastric MALToma
H. Pylori Marginal Zone lymphoma
87
Differences in staging in NHL
Bone marrow biopsy
and
Lumbar puncture
88
Burkitt Lymphoma
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
89
Tx for Burkitt
HyperCVAD regimen
or
R-EPOCH
90
Diffuse Large B-Cell (DLBC)
Most common NHL
extranodal masses- mediastinum
91
Tx for DLBC
R-CHOP
Rituximab
Cyclophos
H (doxorubicin)
O (vincristine)
Prednisone
92
Follicular Lymphoma
T(14;18)
Can transform DLBC
Early stages- radiation alone
93
Tx for later stages of Follicular lymphoma
Rituximab
Or
With Bendamustine
or CVP (Cyclophos,Vincris,Prednisone)
94
Marginal Zone Lymphoma
1. Extranodal marginal zone lymphoma
95
Extranodal marginal zone lymphoma (AKA MALT)
In organs that contain mucosa
1. GI tract
Thyroid, eyes, and lungs
96
MALT tx fail H. Pylori
Radiation
Rituximab or
Chemotherapy
97
Waldenstrom Macroglobulinemia
(AKA lymphoplasmacytic lymphoma)
Excessive IgM
Risk factor- MGUS
98
S/s of walden
Raynaud’s phenomenon (Cold autoimmune hemolytic anemia-IgM)
PLT related bleeding (gums,nose)
Viscosity - requires plasmapheresis
Peripheral Neuropathy
Hepatosplenomegaly/Lymphadenopathy
99
OVA mnemonic for Walden
Organ infiltration
Viscosity
Anemia
100
(SPEP) Serum protein Electrophoresis
Monoclonal spike in gamma region result then need to do immunofixation to see IgM in Walden or IgG/IgA in MM
101
Rouleaux
Stacked coin appearance on peripheral smear seen in both Waldenstrom and MM
102
Tx for Walden
Sxs=
Rituximab or Ibrutinib single agent or with chemo
103
Multiple Myeloma
(Plasma cell myeloma)
IgG- most common
AA
Agent orange
MGUS
104
MM B.R.E.A.K clinical features
Bone pain
Recurrent infections
Elevated calcium
Anemia
Kidney damage - Bence Jones protein
Most because of osteoclast activity
105
Lytic lesions
MM
“Punched out”
Vertebral fractures - back pain
NOT ON BONE SCAN
106
UPEP (urine protein electrophoresis)
M-spike in urine
Bence Jones Protein
107
Tx for MM
2.Auto SCT
1.Combo chemo (VRD or RVD) =
Velcade(Borte), Revlimid*, Dexamethasone
Sxs tx- Bisphosphonates
Plasmacytoma- Radiation
108
MGUS
Lifelong monitoring Q6months
-SPEP/UPEP
-CBC
-BMP
109
Chronic leukemia
Insidious onset
Less aggressive
Mature forms
110
Acute Leukemia
Rapid onset
Aggressive disease
Immature cells (BLASTS forms)
111
AML
Adult pop
Caucasians
Males* MDS risk
Down syndrome
“Chimney sweeps”
112
Sxs of AML
Anemia
Neutropenia
Thrombocytopenia
Bone pain
113
M3 subtype of AML
APL
DIC
114
First clinical presentation of AML
Oncological emergencies-
Neutropenic fever or
Leukostasis
or
Tumor Lysis syndrome
115
Neutropenic fever
ANC <1500
101 F or 100.4 over an hour
116
Tx for Neutropenia fever
Panculture
Broad spectrum antibiotics within 60 min (cover Pseudomonas)
117
Tumor Lysis Syndrome P.U.C.K
Hyper phosphatemia
Hyper uricemia
Hypo calcemia
Hyper kalemia
118
Signs and Sxs of tumor lysis
Decreased urine output
Muscle cramps
N/v
Lethargy
Arrhythmias
AKI
Seizure
100,000 or higher WBC, all Blast cells
119
Tumor lysis tx
Aggressive IVF
Allopurinal or rasburicase (uric acid >14)
Phoslo
Calcium gluconate
120
Leukostasis
Dyspnea!
Altered mental stasis!
HA
Retinal thrombus
Priapism
Dec urine output
121
Leukostasis tx
Leukapheresis first line
122
AML physical findings
Gingival Hyperplasia
Leukemia cutis (more in relapsed Dz)
Granulocytic sarcoma (spleen more common and in subtype M5)
123
Peripheral blood smear in AML/APL
Auer rods
124
Myeloperoxidase
BLASTS stain positive
125
Prior to initiation of AML tx
ECHO - anthracycline
Central line
CMP
126
Prophylactic regimen for AML
Antibiotic- Levaquin
Antiviral- Valtrex
Antifungal- Posaconazole or Fluconazole
127
Induction Chemo for AML
7+3
Cytarabine + Anthracycline
128
Consolidation chemo for AML
HIDAC
High dose cytarabine
129
Acute Promyelocytic Leukemia (APL)
T(15;17)
Younger patients
No SCT
Wet purpura
Fibrinogen less than 125- replace with Cryoprecipitate
130
Tx for APL
ATRA (Vit A) plus chemo or arsenic
131
(ALL) Acute Lymphoblastic leukemia
Most common type of cancer in children- boys
Down syndrome
Previous malignancy
Poorer prognosis in adults
132
T-cell ALL
Teenagers
Thymus enlargement
B-cell is more common
133
ALL s/s
Bone pain more common complaint
Headaches- crosses BBB
Infiltration to extramedullary (ex. lymphadenopathy)
134
(TdT) Terminal deoxynucleotidyl transferase
Positive stain in immature lymphoblasts
135
Prior to tx in ALL
LP
136
Prophylaxis for ALL includes
Bactrim or Dapsone for PCP/PJP ~pneumonia
137
CNS prophylaxis in ALL
Methotrexate or Cytarabine
Hydrocortisone
138
CML
Philadelphia chromosome T(9;22)
Atomic bomb pt
139
S/s of CML
Abdominal fullness
Early satiety
Splenomegaly
140
LAP (leukocyte alkaline phosphatase) Score
CML low Lap score
141
Tx for CML
TKIs first line
Imatinib (gleevac)
Proven cure- Allogenic SCT
142
CLL
Most common leukemia of adults
Lymphadenopathy!
B-cell
143
Isolated lymphocytosis
>5000
CLL
144
Warm autoimmune hemolytic anemia
Sxs- jaundice, tea colored urine
Spherocytes- fat guy in a little suit
Coombs +
IgG
-CLL
145
Smudge cells
Incompetent B cells
CLL!!!!
146
CD19,20,23,5
CLL
147
Venetoclax
CLL tx
148
Polycythemia Vera
Most common MPD
Predominance in RBC’s
HCT >49% in M and >48% in F
JAK2 mutation (not driven by EPO)
149
PV sxs
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
PV Dx
CBC
-WBC shows elevated eosinophils and basophils
LOW EPO Levels
151
Tx for PV
Phlebotomy to HCT <45%
ASA 81 mg
152
What can PV transform to?
AML
or
PM
153
Which gender is ET predominant?
Females
154
Essential thrombocytosis (ET)
PLT >450,000
155
Mutations in ET
JAK2
CALR
MPL
156
Miscarriages in 1st trimester
ET
157
Myelocytes & Metamyelocytes
ET
158
Peripheral smear in ET
Giant PLTs
159
BMBX in ET
Increased megakaryocytes
160
Primary Myelofibrosis (PMF)
Chronic myeloproliferative disorder
Extramedullary hematopoiesis
Enlarged spleen!!!!!!!!!!! CLASSIC
Anemic first
161
Peripheral Smear triad in PMF
1. Leukoerythroblastosis
-nucleated RBC etc
2. Teardrop-shaped RBCs
3. Giant abnormal PLT w/o granules
162
BMBX in PMF
Dry tap
163
What drugs can decrease spleen size in PMF
Hydroxyurea
or
Ruxolitnib (Jakafi)
164
Myelodysplastic syndrome (MDS)
Dysplastic immature cells-nonfunctional
Early apoptosis
165
MDS
Increased incident to transform to AML
Exposure to heavy metals -lead
166
Primary MDS
Idiopathic
Most common
167
Secondary MDS
Chemo/radiation exposure
168
What anemia is found in MDS?
Macrocytic anemia
169
Peripheral blood smear in MDS
Dysplastic RBCs
Hyposegmented neutrophils
170
ANC in MDS
ANC <1800
171
Low risk MDS med management
Azacitidine or Decitabine
172
Hgb F
2 alpha 2 gamma
Highest in utero
173
Hgb A
2 alpha 2 beta
Highest 6 months of age
174
High retic
Hemolysis or bleeding
175
Low retic
Bone marrow issue or
Missing ingredient
176
What medications can raise WBC?
Steroids, Epinephrine
177
What is the most common cause of Leukopenia?
Neutropenia
Severe infection risk ANC <500
178
Anisocytosis
Variation in erythrocyte size
179
Pagophagia, Pica
Iron deficiency anemia
180
Hemolytic anemias
Jaundice
Icterus
Pruritis
Hemoglobinuria
181
COOMBs test
Autoimmune testing
182
What is the most common anemia worldwide?
Iron deficiency anemia- dietary cause
(Blood loss cause in US)
183
Fe deficiency anemia pathognomonic
Ferritin less than 20
184
Hgb A2
2 alpha and 2 delta
185
Thalassemia
Microcytic anemia with normal iron studies
-target cells
Hemolysis labs
186
Hemolysis labs
Haptoglobin -taxi (dec)
LDH (inc)
Indirect bili (inc)
High retic
187
Alpha thalassemia
Southeast Asia and Africa
2 genes defective=
Look at iron studies or will miss dx
188
Hgb H disease
Alpha thalassemia intermedia=
3 genes defective
Splenomegaly
Boney deformities= frontal bossing
Hgb Electrophoresis
189
Hydrops Fetalis
Alpha thalassemia major
4 genes defective
Still borns
190
Beta Thalassemia
2 beta genes on Chromosome 11
Mediterranean origin
191
Beta thalassemia minor
1 gene defective
Increased Hgb A2
192
Beta thalassemia Major: Cooley’s anemia
Hgb F predominant
Transfusion dependent
So need Chelation therapy (Deferasirox)
193
Sideroblastic anemia
More commonly acquired-
Chronic ETOH
Lead
Tx with isoniazid for TB
Vitamin B6 deficiency
Sxs- irritability in children, cog impairment
194
Sideroblastic anemia work up
MCV <80
Basophilic stippling- Lead poisoning
BMBX confirms with “ringed sideroblasts”
Lead Lines in children
195
Sideroblastic anemia tx
Supplementation with Vit B6
Lead poisoning- Succimer
196
Lead level 70 or higher (severe)
Succimer plus IV Calcium disodium EDTA
197
Vitamin B12 (cobalamin) deficiency
Strict vegan diet at risk
Pernicious anemia autoimmune dz is main cause
“Stocking and glove paresthesia”
Macro-Ovalocyte and Hypersegmented =Megablastic anemia
198
Folate deficiency anemia
Alcoholic or Anorexic pts
-methotrexate
Chronic hemolytic anemia
199
Anemia of chronic disease
EPO is suppressed
Hepcidin increases (iron regulator)
TIBC decrease and Ferritin (iron stores) high
Serum iron is decreased
ESR and CRP is elevated
200
Aplastic anemia
Pancytopenia
80% acquired - idiopathic
Autoimmune suppression/destruction by T-cells
Hypoplastic bone marrow
201
BMBX in Aplastic anemia
Hypocellular marrow
202
Severe cases of Aplastic anemia (Adults >40,no BMT donor) tx
ATG (antithymocyte globulin) + Cyclosporin [Both target T cells] and a bone marrow stimulating agent Eltrombopag
203
Hereditary Spherocytosis
Hyperchromic anemia- MCHC elevated
Pigmented gallstones
Coombs neg
204
Confirmation tests for Hereditary Spherocytosis
EMA binding (Eosin-5-maleimide binding test)
205
Tx for Hereditary Spherocytosis
Splenectomy
206
G6PD deficiency
X-linked, males
African and Mediterranean
Stressors like fava beans
Heinz bodies- Bite cells
Episodic hemolysis
207
Sickle cell Anemia
Autosomal recessive disorder that affects beta globin gene
208
Dactylitis
Sickle cell
209
Complication of sickle cell
Avascular necrosis - femoral head(MC)
210
Risk of nonfunctional spleen (by 6 years of age)
Infections from encapsulated bacteria
211
Splenic sequestration crisis
Children, low blood pressure etc
Can be fatal within hours
Aggressive transfusion needed
212
Aplastic Crisis
Sickle cell pt
Most common etiology is Parvovirus-19 (fifth disease)
Need to be hospitalized to watch H & H
213
Howell Jolly bodies
Due to nonfunctional spleen/no spleen
Can be seen by the naked eye
214
Autoimmune Hemolytic Anemia
Warm- females, Lupus,Beta-lactams,
Splenomegaly*
Cold- EBV,Mycoplasma PNA
215
Workup for warm Antibody Hemolytic anemia
MCHC elevated
+ Coombs test
Cold Agglutinin test- Negative
Blood smear can show Spherocytes
216
Tx in Warm
First line Glucocorticoids
2nd line is Rituximab
217
Cold Antibody Hemolytic Anemia
Always normocytic
COOMBs positive for compliment only
Confirmatory test is cold agglutinin titer
Avoid cold or use Rituximab
218
(PNH) Paroxysmal Nocturnal Hemoglobinuria
Acquired mutation
PIGA gene
Decrease of CD55 and CD59 (confirmatory test of flow cytometry) so increased compliment binding
219
PNH triad
Hemolytic anemia
Thrombus in unusual sites
Coca-cola urine (clearer as day progresses)
Sxs- erectile dysfunction and esophageal spasm
220
Tx for PNH
Anti-compliment therapy with
Ravulizumab
