HEME Flashcards
1
Q
Factor V Leiden is in what pathway
A
Common pathway
2
Q
The extrinsic pathway consists of what numbers ?
A
Really just 7 [and 3] that goes down into 10 to 5 to 2 [common pathway]
3
Q
Main symptom of factor 5 Leiden to consider in pathology
A
Unexplained DVT
4
Q
Activated protein C and S inhibit what 2 factors ?
A
5Lieden > and small amount of 8
5
Q
2 important things to remember with protein c and s symptoms ; if they are deficient
A
Neonatal Purpura
Warfarin induced skin necrosis !
6
Q
VTE is affected how in protein C and S deficiency
A
In weird places
At a young age
Recurrent
FAM HX
7
Q
If warfarin is causing necrosis what should you do
A
Stop Warfarin
Admin Vit K
8
Q
ITP often follows what ?
A
Viral infection
9
Q
What are the two types of bleeding assoicated with ITP
A
Dry and Wet bleeds
wet is worse= epistaxis ; gingival bleed
Dry = petechia / brushing
10
Q
When do you give CC for ITP
A
If the platelets are less than 30,000
11
Q
Treatment for thrombotic thyrombocytopenia Purpura
A
Plasma exchange therapy
12
Q
4 sxs to think VWF disease
A
Gingival bleeding [wet]
Heavy menses
Mucocutaneous bleeding
Epistaxis
13
Q
Minor bleeding or procedures treatment in VWF dz
A
Desmopressin
14
Q
Major bleeding or procedures manamgenet in VWF dz
A
VWF concentrate
15
Q
4 reasons to have iron deficiency anemia
A
Chronic bleeding = GI, Menses
Dec iron absorption
GI parasites
Inadequate iron intake
16
Q
3 signs of severe IDA
A
Angular chelitis
Koilynchia
Atrophic glossitis
17
Q
IDA has what level of Ferritin
A
LOW
18
Q
In thalassemia iron levels are what
A
Normal or high
19
Q
2 medications that can cause folate deficiency
A
Methotrexate
Trimethoprim
20
Q
What is homocysteine and mma in Folate deficiency
A
Increased homo normal mma
21
Q
2 foods high in folate to recommend for supplementation
A
Leafy greens, fruit
22
Q
Vitamin B12 deficiency is also called
A
Cobalamin deficiency
23
Q
3 things that can cause B12 deficiency
A
Stomach surgeries = gastrectomy
Pernicious anemia
Chronic alcohol abuse
24
Q
4 examples of neuro sxs that often accompany B12 deficiency
A
Peripheral neuropathy
Diminished sensation
Dementia
Reflex changes
25
Homo and mma for B12 deficiency
Low homo and HIGH MMA
*you gotta be 12 to go to the MMA*
26
Route of B12 management perferred
Parenteral preferred
Can do oral
27
G6PD enzyme is responsible for converting what into what?
NADP+ in to NADPH from glucose
Without this conversion = low glutathione = HIGH FREE RATICALS
Oxidative stress = Hemolytic anemia
28
3 medications that usually cause G6PD pathology
Nitrofurantoin
Malaria drugs
Dapsone
FAVE BEANS DONT FORGET>
29
Why might you have dark urine in G6PD deficiency?
Due to acute hemolytic anemia
30
Positive findings on peripheral smear for RBCs - G6PD
Heinz bodies
Bite or Blister cells
31
Common mutation found in polycythemia Vera
JAK2
32
Polycythemia Vera is an over production of what cells
Myeloid
RBCs // WBCs // Platelets
33
What is the level of EPO assoc with Polycythemia Vera
Decreased!
34
Mainstay treatment plolycythemia Vera
Phlebotomy
35
Sickle cell is what type of genetic disorder
Hbss - Autosomal Recessive
36
What infection can cause a drop in Hb in sickle cell disease
Parvo
37
Positive peripheral smear in sickle cell shows what
Howell Jolly bodies
38
Sickle cell often treated with what
Hydroxyurea
39
When treating with Hydroxyurea make sure to supplement what
Folic acid
40
Thalassemia think what region is affected
Mediterranean
African
41
When does B thalassemia occur
6 months after birth at earliest because fetal blood does not contain the beta chain
42
3 sxs of hemoglobin H disease
Jaundice
Splenomegaly
Hemolytic anemia
43
Type of electrophoresis positives in thalassemia
Target cells !
44
ALL has a proliferation of what
Lymphoblasts
45
ALL effects
All kids
46
3 sxs to key in on ALL
Beta sxs and age
With LAD , hepatosplenomegaly , Petechia
47
Defitnive diagnosis ALL
Bone marrow biopsy with greater 20% lymphoblasts
48
CLL is what
Crushed little lymphocytes - smudge cells
49
CLL occurs in who and what is happening
Monoclonal proliferation of premature B cells
Painless LAD
_ hepatosplenomegaly
B sxs
MC in adults in nutrient rich countries
50
Treatment first line CLL
TKIs
51
AML think what smear
Auer Rods
52
What syndrome may progress to AML
Mylodysplastic syndrome
53
Recurrent infections swollen gums; bimodal distribution think what
AML
54
CML has what two tings
Philly chromosome
TRIPHASIC course = chronic , accelerated , blastic
55
2 things assoc with non Hodgkin lymphoma
Burkitts lymphoma
Previous EBV infection
56
Non hodgkins represents what patho
Malignant transformation of lymphoid [Bs and T’s] into the lymphatic system
57
3 Ps of non Hodgkin’s lymphoma
Painless , persistent , peripheral LAD
W/ B sxs
58
Standard diagnosis and staging for non Hodgkin lymphoma
Lymph node biopsy
Ann Arbor staging
59
Peripheral smear most common in Hodgkin’s lymphoma
Reed sternberg cells “looks like owls”
60
Hodgkins affects what ages?
Bimodal
20 and 50
61
Multiple Myeloma think what 4 things
Lytic lesions
Plasma cells proliferation
B sxs
Bone PAIN
62
3 lab findings in multiple myeloma
Rouleaux formations
Bence jones proteinuria
“M spike” IgG serum electrophoresis
63
Alcoholics generally have what deficiency
Folate
64
What med can help reverse tumor lysis syndrome
Allopurinol
65
Bleeding due to heparin deficiency is treated with what medication
Protamine sulfate
66
Hodgkin lymphoma folks are at increased risk of what
Solid organ malignancy
Cardiovascular disease
67
Intrinsic pathway is what
Extrinsic pathway is what
I = PTT
E = pt
68
HEMOPHILLIA is a concern when? Requires what
Minor trauma causing bruising and effusions
COAG studies
69
Hodgkin’s lymphoma can have what
Retrosternal chest pain
70
With hemochromatosis think
Bronze skin and diabetes
71
Mc cause of pediatric stroke
Sickle cell disease
72
Reversal agent for heparin is ?
Protamine sulfate
73
Heterotrophile antibody helps diagnose what
Mono
74
With ALL think what type of cells
Terminal deoxy neutron tidyl transferase! TDT
Mediastinal mass is super commmmonnnn
75
CLL effects who
Elderly almost exclusively with mature cells
Cause they are OLD
76
Warfarin effects the ____ pathway
Intrinsic ; Pt time
Vit K pathway = factor 2, 7, 9 , 10
77
Helmet cells =
Schistocytes
Thinking TTP
78
Basophilic stipiling occurs in
Thalassemia
Lead or heavy metal
79
Microcytic anemia think
MCV less than 80
Severe IDA
Thalassemias
80
Macrocytic anemia think
MCV greater than 100
Folic acid
Vitamin B12 deficiency
Alcohol abuse; Liver Disease ; Myelodysplastic Syndrome
81
Normocytic anemia think
MCV 80-199
Anemia of chornic disease
Anemia of chronic renal disease
Multifactorial anemia
82
What type of anemia is associated with bone marrow suppression
Aplastic anemia
83
CKD ; Hypothyroidism ; and Low Testoasterone are assoc with
Low levels of trophic factors
84
Think waht with anemia from increased destruction of RBCs
*High reticulocyte count*
Blood low-chronic
Hemolytic anemias : sickle cell, thalassemia ; autoimmune ; drugs
85
Define serum iron; trasferrin; ferritin
Serum iron = free iron
Transferring = binds iron in circulation = TIBC
Ferritin = stores of iron; in liver and endothelial system
86
Koilonychia think what
Spoon nails
IDA
87
3 pathopnuemonic sings found in IDA
Plummer Vinson syndrome = esophageal web formation
Restless Legs
Pica = Iron deficiency syndrome
88
2 basic etiologies of IDA
Increased requirements
Decreased supply
89
where is iron absorbed in the body
Intestinaly —> duodenum
Think disturbed by : intestinal malabsorption like :
-Sprue
-Celiac
-Atrophic Gastritis
90
When do yo recheck ferritin in IDA
6-8 weeks
91
What quantifies sickle cell trait vs. disease
Trait = HbS [heterozygous]
Disease = HbSS [homozygous]
92
What defines alpha and beta thalassemia
Alpha = Alpha 1, Alpha 2 , HbH =3 , Hydrops fetalis = 4 copies of trait.
Beta = Beta Minor vs. Beta Major
93
Sickle cell is what type of genetic inheritance
Autosomal recessive disease
Amino valine is subsided for glutamine to form Bs globin = HbS
Dx = Hemoglobin Electrophoresis
94
Pathophysiology of vaso occlusive episodes in SCD
Combo of
vascular adhesion of sickle cells + trapping of dense sickle cells
95
What happens in SCD with increased in altitude or hypoxia
Splenic infarction
96
2 organs often damaged in sickle cell trait and why
Chronic Micro infarcts lead to kidney and cardiac damage
97
4 common assoc with SCD and chronic hemolysis
Reticulocytosis [3 to 15%]
Unconjugated hyperbililrubinemia
Elevated LDH
Gallstones common!
98
Infectious agents common in SCD
Strep Pneumonia.
H. Influenza
Parvo can cause —> Aplastic crisis
Salmonella typhi—> chronic osteomyelitis
99
3 things that precipitate SCD crisis
Dehydration
Infection
Stress
100
What does acute chest syndrome [SCD] look like on CXR
Perihilar Infiltrate +/- infarcts
TXM = plasmaphoresis
101
What SCD med can reduce frequency of crises
Hydroxyurea
—>Folic acid or Bicarbonate can help
102
B thalessemia changes what part of hemoglobin chain
HbA —> HbF and HbA2 [fetal + less preferred adult chain]
103
MC ethnicity effected by B thalassemia
Italian
Greek
Middle Eastern
104
Genetic variety of b thalassemia minor
Heterozygous condition
105
genetic variety of b thalassemia major
Homozygous condition
ONLY HbF and HbA2
106
Does B thalassemia trait [heterozygous] usually need TXM
NO!
107
What is TXM of choice for B Thalassemia major [homozygous]
Allogenic bone marrow transplant = their own bone marrow.
108
3 common sings of b thalassemia major
Severe micorcytic anemia
Infants develop anemia after first few months when hemoglobin light chains are supposed to change to adult HbA version ; but dont.
Results in cardiac stress[CHF], Hepatosplenomeg; chipmunk facies
109
A thalassemia 2 trait results in :
No abnormalities of MCV or Hb Elecotropheresis
= Loss of only 1/4 alpha globin genes
Think “silent”
110
A thalassemia-1 trait =
Loss of 2/4 alpha globin genes
Mild Anemia ; MCV is less than 80 ; but Hb EP is normal
=B minor
Think A1 “minor”
111
Hemoglobin G disease =
Loss of 3/4 alpha globin genes
Inclusion Bodies in RBCs HbH > predominates
Chronic hemolytic anemia
112
Hydrops fatalis with Hb Barts =
Non of the four alpha globin chains are functional — fatal at birth.
113
Anemia of chronic disease think —>
Chronic inflammation
114
ACD is induced by what two types of cells
Inflammatory cytokines [IL-6]
Hepcidin
115
What happens to iron in ACD
Trapped by macrophages
Decrease in RBC production by BM
EPO is impaired by interferons
Decreased EPO response
116
TIBC in ACD
Low
*high in IDA*
117
Reticulocytes in ACD =
Low
*high in IDA*
118
4 underlying causes of ACD
Acute or Chronic infections
Malignancies
Chronic arthritic conditions-RA ; UC ; Crohns
Thyroid disorders ; DM
119
Most effective txm of ACD
EPO
With Hbg goal of 11-12
Then treat the cause
120
Intracorpuscular defects = and example?
Defect in RBC membrane
—> Hereditary Spherocytosis
121
Extrcorpuscular hemolytic anemia = and examples?
Outside of the RBC causing membrane changes :
G6PD
Autoimmune
Drug effects—> Chemo
122
If hemolytic anemia is autoimmune in origin ; what test will be positive
Coombs Test
123
What management is often required in spherocytosis
Splenectomy
124
RBC morphology for G6Pd deficiency
Bite cells with Heinz bodies
125
Pancytopenia think
Aplastic anemia
126
3 common presentation signs in aplastic anemia
Recurrent infections
Mucosal hemmorhage —> thrombocytopenia
Anemia —> lack of reticulocytes
127
2 drugs and 2 disease that can cause aplastic anemia
Drugs —> Lindane ; Chloramphenicol
Diseases —> EBV ; SLE
128
Alcoholism causes what type of MCV
Megaloblastic
129
ETOH often decreases what vitamin
Folic Acid —> megaloblastic anemia
*think B1 : thiamine
*think Vitamin C : scurvy
130
Neuro symptoms in B12 defiency are likely due to
Lack of cobalamin
131
MMA and Folic Acid in B12 vs. Folate deficiency
B12 = MMA high + homocysteine elevated
Folate = MMA low/nml + homocysteine elevated
132
With pernicious anemia think what 2 things
Autoimmune gastritis attacking intrinsic factor
B12 deficiency —> MCV over 100
133
Describe the mouth in B12 deficiency
Stomatitis
Glossitis
134
Gait description for B12 deficiency
Broad based gait
SLOW REFLEXES
135
Folic acid supplementation in pregnancy is to prevent
Neural tube defects
136
Signs associated with ITP [Idio Thrombo Purpura]
Petechia hemorrhage
Mucosal bleeding
Thrombocytopenia [ less than 20K ]
*bleeding manifests as much less compared to platelet count being so LOW*
137
Management of ITP
Steriods = 1st line ; 4 weeks
IVIG
Splenectomy
138
TTP think what with FAT RN
Fever
Anemia
Thrombocytopenia
Renal impairment [Cr <3]
Neurologic Changes [seizures; AMS]
139
Test findings in TTP
COAG test = normal
ADAMSTS -13 enzyme Ab
140
Factor V Leiden in activated by what protein?
Protein C
141
Platelet vs. Factor type VWF dz
Platelet = heavy menses ; mucosal bleeds; cuts prolong
Factor = deep joint ; post op bleeds
142
Inheritance pattern of VWD
Autosomal dominant
143
Describe pathway and sign lab deficiency in VWF dz
PTT and BT prolonged [ extrinsic ]
Platelet aggregation test - abnormal
144
TXM of VWF dz
Desmopression [DDVAP]
Increases VWF levels by stimulation secretion from endothelium
IV and hour before surgery or major bleeds ; Nasal spray for small bleeding management
145
Hemophilia inheritance pattern
Sex linked recessive
Gene on long arm of X chromosome
146
Hemophilia A think [4]
MALE
PTT prolonged only;
Mild TXM = DDVAP ; Severe TXM = IV Factor 8
Hemarthrosis ; soft tissue bleeding ; mucosal bleeding
147
Hemophilia B think [4]
Factor 9 deficient
Christmas Disease
TXM = recombinant Factor 9 concentrate
Hemarthrosis ; blood in stools ; nosebleeds
148
Factor V Leiden think [3]
Unopposed coagulation pathway
Heterozygous = + 1st TE = AC 6-12 months ; 2nd TE = lifelong AC
Homozygous = lifelong AC
149
AT3 deficiency would black what pathways and think what drug
Blocks 2. 7. 9. 10. 12. 11.
Think heparin .
1st Provoked TE = 6 mos AC
Unprovoked TE = lifelong
150
Protein C and S deficiency would block
Factor 5 and Factor 8 = rapid thrombin formation
Only treat if thrombosis risk = high
151
Increased prothrombin would cause
Heterozygous ; 1st event = 6-12months AC TXM
Homozygous ; 2nd event = lifelong AC TXM
152
PCV is due to
Hypoxemia
Think : OSA and Smokers
153
2 findings diagnostic for PCV
JAK2 mutation and EPO level low
154
PCV has decreased prognosis if transformed to what disease
Myelofibrosis
155
3 TXMs helpful for PCV
1st line = therapeutic phlebotomy ; Hydroxyurea
ASA
156
Platelet count often found in essential thrombocytosis
450K —> 1000K platelets
Increases without stimulation
+JAK2 ; CALR ; MPL mutations+
157
Hemochromatosis defintion
Abnormal absorption of iron with increased storage in organs
1st = high iron and fatigue
Progresses = cirrhosis ; HCC ; DM ; CHF
TXM = phlebotomy to keep ferritin less 50
158
Genetic mutation in precursor cells in marrow
Cytopenia in any or all cell lines
MACROCYTOSIS
Monocytosis
Myelodysplastic syndrome
159
Acute lymphocytic leukemia [4]
ALL KIDS
RF in Adults : radiation ; chemo ; organic solvents ; Down syndrome
Sxs : infections; bleeding ; LAD; HSM; high WBCs
TXT = chemo ; kids =good prognosis ; adults = bad
160
Acute myeloid leukemia [4]
Peak age = 60 yrs old
Greater 20% blasts or immature cells —> BM dz sxs + chloroma = tissue infiltrate // leukemia cutis = skin lesions
+Auer Rods = aggregates of myeloid granules
TXM = Chemo
161
Chronic lymphocytic leukemia
Age = 62 yrs old ; most common in WEstern countries
+Smudge cells with lymphocytosis
Sxs = severe dz = HSM ; increased doubling time ; recurrent infections ; cell line depletion
TXM = TKI = Imbruvica ; radiation ; immune therapy ; chemo
162
Chronic myeloenous leukemia
+philly chromosome —> 22 gene translocation mutation
Remain in chronic phase for years —> accelerated phase [10-19% blasts ; low plts/Hbg] —> blast crisis = above 20% blasts [death = weeks to months]
Found in peripheral blood flow cytometry
TXM = imatinib with 80% remission ;; ADE = fluid overload and cardiac events
163
Multiple Myeloma think what findings ? [CRAB]
Calcium elevated
Renal insufficiency
Anemia = BM suppression
Bone = Lytic bone lesions
164
Protein / Albumin in MM
Large variance
165
Stepwise findings in MM
MGUS —> Smoldering Myeloma —> MM
[no crab / less 10% plasma cells in marrow] —> MGUS
[10-60% plasma cells in marrow = M SPIKE] —> SMOLDERING MYELOMA
[greater 60% plasma cells in marrow / 10% with CRAB] —> MM
166
Clinically stable MM TXM of choice
Stem cell transplant ;
If cant = cc ; chemo
167
4 complications of MM
Cord compression
Hypercalcemia
Renal F.
Hyper viscosity
168
NHL presentation
Weight loss
Painless LAD
Night sweats / FEVER
Pruritis
Chest ABD Pain
169
Mangement NHL
Chemo / Radiation
If CD20+ = Rituximab
170
NHL think
Painless LAD
171
8 non lymph organs? vs. primary lymph organs = bone marrow and thymus.
Stomach
Tonsils
Spleen
Intestines
Lungs
Liver
Bones
Skin
172
Hodkins Lymphoma [5]
MALE bimodal age distro ; HLA Link
Painless lymph node with enlargement [head and neck] + B sxs
Post ETOH = painFUL Lymph nodes
Dx = + Reed STERNBERG biopsy ; Mediastinal involvement common
TXM = CHEMO [ABVD]
SCT for refractory
173
TRALI management
Stop transfusion
O2 supplementation
Ventilatory support
174
Management of volume overload in Transfusion of CHF ; CKD patient
Slower infusion rate
Diuretics +
175
Post transfusion Purpura presents
1-2 weeks after transfusion with bleeding
Self limited process bit in severe cases TXM = IVIG and CC
176
What is an example of an iron chelating agent
Desfuroxime / deferasirox
177
How do you dx graft vs hosts disease
Check circulating lymphocyte HLA against host tissue HLA
178
Alpha globin thalessemia is common in what ethnicity
Asians
179
B12 is a cofactor in conversion of what
Homocysteine to methionine
180
SCD often presents as what type of anemia
Hemolytic
181
Hemochromatosis folks should avoid what ? [4]
Alcohol
Vitamin C
Raw shellfish
Supplemental Iron
182
What type of lymphoma presents with pruritus
Hodgkins Lymphome + Reed STERNBERG in lymph node bx
183
MM can be confirmed by what diagnostic
Serum protein electrophoresis
184
Beta thalassemia major will have sxs presenting when
At 6 months of life
185
Non hodkins Lymphoma is dx by
Biopsy = tissue or Lymph node
186
IDA skin findings
Chelosis
Brittle nails
Smooth tongue
Koilynchia
187
Beta thalassemia physical signs
Bony deformities
Hepatosplenomegaly
188
B12 deficiency physical signs
Glossitis
Decreased vibration / position sens
Difficult balance
189
Aplastic anemia physical signs
Pupura
Petechia
Pallor
190
PCV physical signs
Plethora
Splenomegaly
Engorged retinal veins
191
ITP treatment
Predisone
IV IG or Anti-D
192
Cornerstone of TXM for acute SCD with stroke sxs
Blood transfusions
193
Types of stroke in adult vs. child SCD
Adult = hemorrhagic
Kids = ischemic
194
Autoimmune hemolytic anemia management
Blood transfusions
+
Rituximab
+
CC
195
What drug commonly induces autoimmune hemolytic anemia
Methotrexate
196
3 most common risks for CN are that are modifiable
Tobacco
Lack of physical activity
Dietary intake
197
What is erythromelagia and what is its associated disease
= painful biting of the hands accompanied by erythema
Dz = essential thrombocytosis
198
Hemochromatosis think
BRONZE diabetes ; new onset
199
What is the name of lymphoblasts in ALL
Terminal deoxynucleotidyl transferase
200
What is the reversal agent for heparin
Protamine sulfate
201
reversal agents for warfarin
Vitamin K
FFP
Prothrombin complex concentrate = replaces factors 2;7;9;10
202
Medications that trigger hemolysis in G6PD [5]
Dapsone
Nitrofurantoin
Primaquine
Rasburicase
Isobutyl nitrate
203
VWF dz affects what part of the bleeding
PTT = prolonged = extrinsic
204
HUS has a decreased what
Platelet count
205
HIT management
With : stop heparin and give Facotr 10a
206
Common complication of DIC and what do the cells look like
Bacterial meningitis = gram negative diplocci
+shistocytes
Decreased fibrinogen
Prolonged PT and PTT
207
2 deficiencies associated with phenytoin use
Gingival hyperplasia
+
Folic acid deficiency
