HEME Flashcards
Factor V Leiden is in what pathway
Common pathway
The extrinsic pathway consists of what numbers ?
Really just 7 [and 3] that goes down into 10 to 5 to 2 [common pathway]
Main symptom of factor 5 Leiden to consider in pathology
Unexplained DVT
Activated protein C and S inhibit what 2 factors ?
5Lieden > and small amount of 8
2 important things to remember with protein c and s symptoms ; if they are deficient
Neonatal Purpura
Warfarin induced skin necrosis !
VTE is affected how in protein C and S deficiency
In weird places
At a young age
Recurrent
FAM HX
If warfarin is causing necrosis what should you do
Stop Warfarin
Admin Vit K
ITP often follows what ?
Viral infection
What are the two types of bleeding assoicated with ITP
Dry and Wet bleeds
wet is worse= epistaxis ; gingival bleed
Dry = petechia / brushing
When do you give CC for ITP
If the platelets are less than 30,000
Treatment for thrombotic thyrombocytopenia Purpura
Plasma exchange therapy
4 sxs to think VWF disease
Gingival bleeding [wet]
Heavy menses
Mucocutaneous bleeding
Epistaxis
Minor bleeding or procedures treatment in VWF dz
Desmopressin
Major bleeding or procedures manamgenet in VWF dz
VWF concentrate
4 reasons to have iron deficiency anemia
Chronic bleeding = GI, Menses
Dec iron absorption
GI parasites
Inadequate iron intake
3 signs of severe IDA
Angular chelitis
Koilynchia
Atrophic glossitis
IDA has what level of Ferritin
LOW
In thalassemia iron levels are what
Normal or high
2 medications that can cause folate deficiency
Methotrexate
Trimethoprim
What is homocysteine and mma in Folate deficiency
Increased homo normal mma
2 foods high in folate to recommend for supplementation
Leafy greens, fruit
Vitamin B12 deficiency is also called
Cobalamin deficiency
3 things that can cause B12 deficiency
Stomach surgeries = gastrectomy
Pernicious anemia
Chronic alcohol abuse
4 examples of neuro sxs that often accompany B12 deficiency
Peripheral neuropathy
Diminished sensation
Dementia
Reflex changes
Homo and mma for B12 deficiency
Low homo and HIGH MMA
you gotta be 12 to go to the MMA
Route of B12 management perferred
Parenteral preferred
Can do oral
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
3 medications that usually cause G6PD pathology
Nitrofurantoin
Malaria drugs
Dapsone
FAVE BEANS DONT FORGET>
Why might you have dark urine in G6PD deficiency?
Due to acute hemolytic anemia
Positive findings on peripheral smear for RBCs - G6PD
Heinz bodies
Bite or Blister cells
Common mutation found in polycythemia Vera
JAK2
Polycythemia Vera is an over production of what cells
Myeloid
RBCs // WBCs // Platelets
What is the level of EPO assoc with Polycythemia Vera
Decreased!
Mainstay treatment plolycythemia Vera
Phlebotomy
Sickle cell is what type of genetic disorder
Hbss - Autosomal Recessive
What infection can cause a drop in Hb in sickle cell disease
Parvo
Positive peripheral smear in sickle cell shows what
Howell Jolly bodies
Sickle cell often treated with what
Hydroxyurea
When treating with Hydroxyurea make sure to supplement what
Folic acid
Thalassemia think what region is affected
Mediterranean
African
When does B thalassemia occur
6 months after birth at earliest because fetal blood does not contain the beta chain
3 sxs of hemoglobin H disease
Jaundice
Splenomegaly
Hemolytic anemia
Type of electrophoresis positives in thalassemia
Target cells !
ALL has a proliferation of what
Lymphoblasts
ALL effects
All kids
3 sxs to key in on ALL
Beta sxs and age
With LAD , hepatosplenomegaly , Petechia
Defitnive diagnosis ALL
Bone marrow biopsy with greater 20% lymphoblasts
CLL is what
Crushed little lymphocytes - smudge cells
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
Treatment first line CLL
TKIs
AML think what smear
Auer Rods
What syndrome may progress to AML
Mylodysplastic syndrome
Recurrent infections swollen gums; bimodal distribution think what
AML
CML has what two tings
Philly chromosome
TRIPHASIC course = chronic , accelerated , blastic
2 things assoc with non Hodgkin lymphoma
Burkitts lymphoma
Previous EBV infection
Non hodgkins represents what patho
Malignant transformation of lymphoid [Bs and T’s] into the lymphatic system
3 Ps of non Hodgkin’s lymphoma
Painless , persistent , peripheral LAD
W/ B sxs
Standard diagnosis and staging for non Hodgkin lymphoma
Lymph node biopsy
Ann Arbor staging
Peripheral smear most common in Hodgkin’s lymphoma
Reed sternberg cells “looks like owls”
Hodgkins affects what ages?
Bimodal
20 and 50
Multiple Myeloma think what 4 things
Lytic lesions
Plasma cells proliferation
B sxs
Bone PAIN
3 lab findings in multiple myeloma
Rouleaux formations
Bence jones proteinuria
“M spike” IgG serum electrophoresis
Alcoholics generally have what deficiency
Folate
What med can help reverse tumor lysis syndrome
Allopurinol
Bleeding due to heparin deficiency is treated with what medication
Protamine sulfate
Hodgkin lymphoma folks are at increased risk of what
Solid organ malignancy
Cardiovascular disease
Intrinsic pathway is what
Extrinsic pathway is what
I = PTT
E = pt
HEMOPHILLIA is a concern when? Requires what
Minor trauma causing bruising and effusions
COAG studies
Hodgkin’s lymphoma can have what
Retrosternal chest pain
With hemochromatosis think
Bronze skin and diabetes
Mc cause of pediatric stroke
Sickle cell disease
Reversal agent for heparin is ?
Protamine sulfate
Heterotrophile antibody helps diagnose what
Mono
With ALL think what type of cells
Terminal deoxy neutron tidyl transferase! TDT
Mediastinal mass is super commmmonnnn
CLL effects who
Elderly almost exclusively with mature cells
Cause they are OLD
Warfarin effects the ____ pathway
Intrinsic ; Pt time
Vit K pathway = factor 2, 7, 9 , 10
Helmet cells =
Schistocytes
Thinking TTP
Basophilic stipiling occurs in
Thalassemia
Lead or heavy metal
Microcytic anemia think
MCV less than 80
Severe IDA
Thalassemias
Macrocytic anemia think
MCV greater than 100
Folic acid
Vitamin B12 deficiency
Alcohol abuse; Liver Disease ; Myelodysplastic Syndrome
Normocytic anemia think
MCV 80-199
Anemia of chornic disease
Anemia of chronic renal disease
Multifactorial anemia
What type of anemia is associated with bone marrow suppression
Aplastic anemia
CKD ; Hypothyroidism ; and Low Testoasterone are assoc with
Low levels of trophic factors
Think waht with anemia from increased destruction of RBCs
High reticulocyte count
Blood low-chronic
Hemolytic anemias : sickle cell, thalassemia ; autoimmune ; drugs
Define serum iron; trasferrin; ferritin
Serum iron = free iron
Transferring = binds iron in circulation = TIBC
Ferritin = stores of iron; in liver and endothelial system
Koilonychia think what
Spoon nails
IDA
3 pathopnuemonic sings found in IDA
Plummer Vinson syndrome = esophageal web formation
Restless Legs
Pica = Iron deficiency syndrome
2 basic etiologies of IDA
Increased requirements
Decreased supply
where is iron absorbed in the body
Intestinaly —> duodenum
Think disturbed by : intestinal malabsorption like :
-Sprue
-Celiac
-Atrophic Gastritis
When do yo recheck ferritin in IDA
6-8 weeks
What quantifies sickle cell trait vs. disease
Trait = HbS [heterozygous]
Disease = HbSS [homozygous]
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
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
Pathophysiology of vaso occlusive episodes in SCD
Combo of
vascular adhesion of sickle cells + trapping of dense sickle cells
What happens in SCD with increased in altitude or hypoxia
Splenic infarction
2 organs often damaged in sickle cell trait and why
Chronic Micro infarcts lead to kidney and cardiac damage
4 common assoc with SCD and chronic hemolysis
Reticulocytosis [3 to 15%]
Unconjugated hyperbililrubinemia
Elevated LDH
Gallstones common!
Infectious agents common in SCD
Strep Pneumonia.
H. Influenza
Parvo can cause —> Aplastic crisis
Salmonella typhi—> chronic osteomyelitis
3 things that precipitate SCD crisis
Dehydration
Infection
Stress
What does acute chest syndrome [SCD] look like on CXR
Perihilar Infiltrate +/- infarcts
TXM = plasmaphoresis
What SCD med can reduce frequency of crises
Hydroxyurea
—>Folic acid or Bicarbonate can help
B thalessemia changes what part of hemoglobin chain
HbA —> HbF and HbA2 [fetal + less preferred adult chain]
MC ethnicity effected by B thalassemia
Italian
Greek
Middle Eastern
Genetic variety of b thalassemia minor
Heterozygous condition
genetic variety of b thalassemia major
Homozygous condition
ONLY HbF and HbA2
Does B thalassemia trait [heterozygous] usually need TXM
NO!
What is TXM of choice for B Thalassemia major [homozygous]
Allogenic bone marrow transplant = their own bone marrow.
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
A thalassemia 2 trait results in :
No abnormalities of MCV or Hb Elecotropheresis
= Loss of only 1/4 alpha globin genes
Think “silent”
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”
Hemoglobin G disease =
Loss of 3/4 alpha globin genes
Inclusion Bodies in RBCs HbH > predominates
Chronic hemolytic anemia
Hydrops fatalis with Hb Barts =
Non of the four alpha globin chains are functional — fatal at birth.
Anemia of chronic disease think —>
Chronic inflammation
ACD is induced by what two types of cells
Inflammatory cytokines [IL-6]
Hepcidin
What happens to iron in ACD
Trapped by macrophages
Decrease in RBC production by BM
EPO is impaired by interferons
Decreased EPO response
TIBC in ACD
Low
high in IDA
Reticulocytes in ACD =
Low
high in IDA
4 underlying causes of ACD
Acute or Chronic infections
Malignancies
Chronic arthritic conditions-RA ; UC ; Crohns
Thyroid disorders ; DM
Most effective txm of ACD
EPO
With Hbg goal of 11-12
Then treat the cause
Intracorpuscular defects = and example?
Defect in RBC membrane
—> Hereditary Spherocytosis
Extrcorpuscular hemolytic anemia = and examples?
Outside of the RBC causing membrane changes :
G6PD
Autoimmune
Drug effects—> Chemo
If hemolytic anemia is autoimmune in origin ; what test will be positive
Coombs Test
What management is often required in spherocytosis
Splenectomy
RBC morphology for G6Pd deficiency
Bite cells with Heinz bodies
Pancytopenia think
Aplastic anemia
3 common presentation signs in aplastic anemia
Recurrent infections
Mucosal hemmorhage —> thrombocytopenia
Anemia —> lack of reticulocytes
2 drugs and 2 disease that can cause aplastic anemia
Drugs —> Lindane ; Chloramphenicol
Diseases —> EBV ; SLE
Alcoholism causes what type of MCV
Megaloblastic
ETOH often decreases what vitamin
Folic Acid —> megaloblastic anemia
*think B1 : thiamine
*think Vitamin C : scurvy
Neuro symptoms in B12 defiency are likely due to
Lack of cobalamin
MMA and Folic Acid in B12 vs. Folate deficiency
B12 = MMA high + homocysteine elevated
Folate = MMA low/nml + homocysteine elevated
With pernicious anemia think what 2 things
Autoimmune gastritis attacking intrinsic factor
B12 deficiency —> MCV over 100
Describe the mouth in B12 deficiency
Stomatitis
Glossitis
Gait description for B12 deficiency
Broad based gait
SLOW REFLEXES
Folic acid supplementation in pregnancy is to prevent
Neural tube defects
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
Management of ITP
Steriods = 1st line ; 4 weeks
IVIG
Splenectomy
TTP think what with FAT RN
Fever
Anemia
Thrombocytopenia
Renal impairment [Cr <3]
Neurologic Changes [seizures; AMS]
Test findings in TTP
COAG test = normal
ADAMSTS -13 enzyme Ab
Factor V Leiden in activated by what protein?
Protein C
Platelet vs. Factor type VWF dz
Platelet = heavy menses ; mucosal bleeds; cuts prolong
Factor = deep joint ; post op bleeds
Inheritance pattern of VWD
Autosomal dominant
Describe pathway and sign lab deficiency in VWF dz
PTT and BT prolonged [ extrinsic ]
Platelet aggregation test - abnormal
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
Hemophilia inheritance pattern
Sex linked recessive
Gene on long arm of X chromosome
Hemophilia A think [4]
MALE
PTT prolonged only;
Mild TXM = DDVAP ; Severe TXM = IV Factor 8
Hemarthrosis ; soft tissue bleeding ; mucosal bleeding
Hemophilia B think [4]
Factor 9 deficient
Christmas Disease
TXM = recombinant Factor 9 concentrate
Hemarthrosis ; blood in stools ; nosebleeds
Factor V Leiden think [3]
Unopposed coagulation pathway
Heterozygous = + 1st TE = AC 6-12 months ; 2nd TE = lifelong AC
Homozygous = lifelong AC
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
Protein C and S deficiency would block
Factor 5 and Factor 8 = rapid thrombin formation
Only treat if thrombosis risk = high
Increased prothrombin would cause
Heterozygous ; 1st event = 6-12months AC TXM
Homozygous ; 2nd event = lifelong AC TXM
PCV is due to
Hypoxemia
Think : OSA and Smokers
2 findings diagnostic for PCV
JAK2 mutation and EPO level low
PCV has decreased prognosis if transformed to what disease
Myelofibrosis
3 TXMs helpful for PCV
1st line = therapeutic phlebotomy ; Hydroxyurea
ASA
Platelet count often found in essential thrombocytosis
450K —> 1000K platelets
Increases without stimulation
+JAK2 ; CALR ; MPL mutations+
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
Genetic mutation in precursor cells in marrow
Cytopenia in any or all cell lines
MACROCYTOSIS
Monocytosis
Myelodysplastic syndrome
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
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
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
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
Multiple Myeloma think what findings ? [CRAB]
Calcium elevated
Renal insufficiency
Anemia = BM suppression
Bone = Lytic bone lesions
Protein / Albumin in MM
Large variance
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
Clinically stable MM TXM of choice
Stem cell transplant ;
If cant = cc ; chemo
4 complications of MM
Cord compression
Hypercalcemia
Renal F.
Hyper viscosity
NHL presentation
Weight loss
Painless LAD
Night sweats / FEVER
Pruritis
Chest ABD Pain
Mangement NHL
Chemo / Radiation
If CD20+ = Rituximab
NHL think
Painless LAD
8 non lymph organs? vs. primary lymph organs = bone marrow and thymus.
Stomach
Tonsils
Spleen
Intestines
Lungs
Liver
Bones
Skin
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
TRALI management
Stop transfusion
O2 supplementation
Ventilatory support
Management of volume overload in Transfusion of CHF ; CKD patient
Slower infusion rate
Diuretics +
Post transfusion Purpura presents
1-2 weeks after transfusion with bleeding
Self limited process bit in severe cases TXM = IVIG and CC
What is an example of an iron chelating agent
Desfuroxime / deferasirox
How do you dx graft vs hosts disease
Check circulating lymphocyte HLA against host tissue HLA
Alpha globin thalessemia is common in what ethnicity
Asians
B12 is a cofactor in conversion of what
Homocysteine to methionine
SCD often presents as what type of anemia
Hemolytic
Hemochromatosis folks should avoid what ? [4]
Alcohol
Vitamin C
Raw shellfish
Supplemental Iron
What type of lymphoma presents with pruritus
Hodgkins Lymphome + Reed STERNBERG in lymph node bx
MM can be confirmed by what diagnostic
Serum protein electrophoresis
Beta thalassemia major will have sxs presenting when
At 6 months of life
Non hodkins Lymphoma is dx by
Biopsy = tissue or Lymph node
IDA skin findings
Chelosis
Brittle nails
Smooth tongue
Koilynchia
Beta thalassemia physical signs
Bony deformities
Hepatosplenomegaly
B12 deficiency physical signs
Glossitis
Decreased vibration / position sens
Difficult balance
Aplastic anemia physical signs
Pupura
Petechia
Pallor
PCV physical signs
Plethora
Splenomegaly
Engorged retinal veins
ITP treatment
Predisone
IV IG or Anti-D
Cornerstone of TXM for acute SCD with stroke sxs
Blood transfusions
Types of stroke in adult vs. child SCD
Adult = hemorrhagic
Kids = ischemic
Autoimmune hemolytic anemia management
Blood transfusions
+
Rituximab
+
CC
What drug commonly induces autoimmune hemolytic anemia
Methotrexate
3 most common risks for CN are that are modifiable
Tobacco
Lack of physical activity
Dietary intake
What is erythromelagia and what is its associated disease
= painful biting of the hands accompanied by erythema
Dz = essential thrombocytosis
Hemochromatosis think
BRONZE diabetes ; new onset
What is the name of lymphoblasts in ALL
Terminal deoxynucleotidyl transferase
What is the reversal agent for heparin
Protamine sulfate
reversal agents for warfarin
Vitamin K
FFP
Prothrombin complex concentrate = replaces factors 2;7;9;10
Medications that trigger hemolysis in G6PD [5]
Dapsone
Nitrofurantoin
Primaquine
Rasburicase
Isobutyl nitrate
VWF dz affects what part of the bleeding
PTT = prolonged = extrinsic
HUS has a decreased what
Platelet count
HIT management
With : stop heparin and give Facotr 10a
Common complication of DIC and what do the cells look like
Bacterial meningitis = gram negative diplocci
+shistocytes
Decreased fibrinogen
Prolonged PT and PTT
2 deficiencies associated with phenytoin use
Gingival hyperplasia
+
Folic acid deficiency
