Hematology/Oncology Flashcards
Main causes of microcytic hypochromic anemia (what is the most common?)
- MC is iron deficiency anemia
- Thalassemia minor/major
- Anemia of Chronic Disease/inflammatory diseases
- Sideroblastic Anemias and Pb Poisoning
Iron deficiency anemia accounts for ____% of anemias worldwide
50%
Normal lab values for the following:
- -Serum Iron, Fe
- -Total Iron Binding Capacity
- -Fe/TIBC saturation
- -Serum Ferritin
Serum Iron: 40-140 uG/dL
TIBC: 200-400 uG/dL
Fe/TIBC Saturation: 25-50%
Serum Ferritin: 30-250 ng/ml
Causes of Iron Deficiency Anemia
- GI Bleeding (ALWAYS BE SUSPICIOUS OF THIS!)
- Excessive menstruation
- Malnutrition & Dietary insufficiency
- Malabsorption (celiac sprue, crohn’s, subtotal gastrectomy)
- Increased demand (pregnancy, growth spurts in children)
- Blood donation, bllod loss in dialysis & Factious Auto-phlebotomy
Specific clinical manifestations of iron deficiency anemia
- -Angular Cheilosis
- -PICA
- -Koilonychia
- -Plummer-Vinson Syndrome (Fe def anemia, esophageal webs, dysphagia and atrophic glossitis)
What do Fe levels, TIBC, saturation, and ferritin look like in Iron Deficiency Anemia
Dec. Fe++
Increase TIBC
Dec. Saturation < 10%
Dec. Ferritin < 20 ng/dL
Tx of choice for iron deficiency anemia
Ferrous sulfate 325mg TID w/ meals for 6 months
Can give IV/IM doses if pt is not oral tolerant
Fe++ side effects
- Constipation
- Black stools
- Nausea
- Bloating
- Abdominal Pain
- Diarrhea
In which drugs does Fe bind to and decrease their activity?
Tetracyclines
Fluroquinolones (cipro, levofloxin)
______ increases Fe++ absorption
Vitamin C (orange juice)
Does AML typically appear in the older or younger population?
Older (>65)
Most common signs/symptoms in AML
< 3 month onset
Fever, bleeding, fatigue, SOB
Less common: retinal hemorrhages, gingival hyperplasia, solid tumors
Polys and blast cells with nothing inbetween is _______ and is a sign for _______.
Leukemic Hiatus
AML
Auer Rods are characteristic cells for what?
AML
How do you treat AML?
Induction therapy
- -> 7 days of Cytarabine continuous IV
- -> 3 days of anthracycline
If not entered CR then retreat or switch to salvage therapy
Older pts >70 do not tolerate standard therapy…need supportive therapy if they chose to go through this (abx, blood products, etc.)
A patient presents with a fever, fatigue, shortness of breath w/ bleeding and thrombosis. What is the likely diagnosis?
APL (Acute Promyelocytic Leukemia)
Name the 5 clotting factors and the 5 anti-clotting factors
Clotting: Platelets, soluble clotting factors, tissue factor, collagen, phospholipids
Anti-clotting: Nitric Oxide, protein C & S, Anti-thrombin III, fibrinolytics, prostacyclin
2 vWF functions
- Binds factor VIII and extends its life
2. Binds platelets to injured vessel endothelium
What does Protime (PT) measure and what is its usual length
Factors II, VII, IX, X
~12 seconds
Is a PT shortened or prolonged when a pt is taking Coumadin?
Prolonged
What does aPTT measure and what is its usual length?
Factors II (prothrombin), VIII, IX, X, XI & Fibrinogen
~32 seconds
Where is vWF stored?
Epithelial cells
Platelets
vWF’s production is stimulated by ______ & ______ and its release is stimulated by _____, _____, ______, & ______.
Production: estrogen & T4
Release: vasopressin, epinephrine, histamine, thrombin
Hemophilia A is a deficiency of factor ___ and Hemophilia B is a deficiency of factor ____.
A = Factor VIII
B = Factor IX
Which type of vWF Disease is more common? Type 1 or Type 2?
Type 1
How do you treat vWF disease type 1 vs. type 2?
Type 1 = DDAVP as nasal spray
Type 2 = DO NOT give DDAVP because it can cause thrombocytopenia
Describe the inheritence of vWF, Hemophilia A&B, and Factor XI Deficiency
vWF = autosomal co-dominant
Hemophilia A&B = x-linked recessive
Factor XI Deficiency = autosomal co-dominant
Which is more common? Hemophilia A or B?
A: 1 in 5000
B: 1 in 30,000
_____ affects 6% of Jewish population.
Factor XI Deficiency
Tx for Factor XI Deficiency
FFP
What is Vitamin K responsible for activating?
F II F VII F XI F X Proteins C & S
Half life of Factor VII is ____.
Half life of Prothrombin is ___.
Half life of Factor X is ____.
VII = 4 hrs Prothrombin = 72 hours X = 48 hrs
Therapeutic Coumadin level for PT and INR
PT = 17-25 INR = 1.5-3
1st line tx for coagulopathy of liver disease
FFP
1st line tx for coagulopathy of kidney disease
Restore renal function w/ dialysis
One pt has coagulopathy of the liver and the other has one of the kidney. Which will likely be more severe?
Liver
Where is DIC most commonly observed?
Septic shock
Underlying conditions of DIC
- Snake or insect venom
- Bacteremia/Sepsis
- Neoplasms (adenocarcinomas, APL)
- Brain Injury
- Fat or Amniotic fluid emboli
- Abrutio placentae
- Severe pre-eclampsia
- HELLP Syndrome
HELLP Syndrome
Hemolysis
Elevated LFTs
Low Platelets
Clinical Hallmark of DIC
Diffuse bleeding from mucosal sites, sites of previous surgery
Organ failure (kidney and liver most common)
Lab values for DIC:
- -Blood work?
- -PT & aPTT
Micro-angiopathic Hemolytic Anemia
Thrombocytopenia
Inc. PT and aPTT time
Tx for DIC
–first line and what to do after
Treat the underlying cause
Next think about replacing clotting and anti-clotting factors w/ FFP
No platelet tranfusions or anti-coagulants
How would you check to see if a patient has developed auto-antibodies to inhibit clotting factors?
1:1 Normal Plasma Mixing Study
- -> Mix pt’s plasma w/ normal plasma and rechecking the abnormal clotting studies (PT & aPTT). If bleeding is d/t a factor deficiency then the normal plasma will correct the problem and the PT and aPTT will be normal.
- -> If the bleeding is d/t an inhibitor then the inhibitor will anticoagulate the clotting factors in the normal plasma and the PT and aPTT will remain abnormal
How do you tx one who has acquired auto -antibodies inhibiting factor VIII?
High dose of Factor VIII replacement along w/ immune suppressive rx
The 3 types of drugs that inhibit platelet function
- ) Aspirin
- ) Clopidogrel
- ) GPIIb/GPIIIa Inhibitors (abciximab, eptifiatide)
Does Aspirin irreversibly bind or reversibly bind to platelets?
Irreversibly…so it lasts for the life of a platelet (7 days)
T/F: High does of platelets are effective to use as anti-platelet rx
False
Dose: 81-325mg
The most commonly inherited hypercoaguable state is:
Factor V Leiden
Tx for Factor V Leiden
Asymptomatic carriers = education about increased risk situations
In high risk situations = rx w/ anti-coagulation until situation is resolved
If pt has > 1 previous DVT or PE = anticoagulation for life
The 2nd most commonly inherited hypercoaguable state that increases plasma levels of prothrombin is _________.
Prothrombin Gene Mutation G20210A
A hypercoaguable state that is often associated w/ SLE
The Anti-Phospholipid Antibody Syndrome
Clotting problems are d/t these 5 reasons:
- Problems of vessel walls
- Problems w/ soluble clotting factors
- Problems w/ soluble anti-clotting factors
- Problem w/ platelets
- Problems w/ a mixture of components of the clotting system
Classic Pentad of TTP and HUS
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Renal Failure
- Fluctuating Neuro s/s
- Fever
If a pt presents w/ the TTP& HUS pentad of symptoms but renal failure is the dominating one is the diagnosis most likely TTP or HUS?
HUS
Which is more common in children? TTP or HUS?
HUS
Describe the following lab diagnosis for TTP:
- -Type of anemia
- -PT and aPTT
- -LDH
- -Indirect Bilirubin
- -Haptoglobin
- -Reticulocyte count
- -Direct Coombs
Microangiopathic Hemolytic Anemia w/ thrombocytopenia
Normal PT and aPTT
Increased LDH Inc. Indirect Bilirubin Dec. Haptoglobin Inc. retic's Negative Direct Coombs
Treatment for TTP and HUS
Urgent plasmapharesis
If offending agent is identified then discontinue it
Steroids may be of some benefit
Before plasmaphoresis, TTP had an 80-90% mortality rate but now if prompt diagnosis and tx are initating it should be ____.
Relapses occur in _____% of cases so long term _____ may be used.
Is DIC a syndrome or a disease?
A syndrome
What must one exclude to diagnose someone w/ ITP?
Lymphomas Medications SLE HIV Hepatitis B or C vWD Antiphosphlipid Syndrome
ITP w/ autoimmune hemolytic anemia is ______.
Evans Syndrome
Myeloproliferative Syndromes:
- -Too many RBC =
- -Too many platelets =
- -Too many neutrophils =
- -Too many RBC, platelets, neutrophis =
Polycythemia Vera (PVC)
Essential thrombocytosis (ET)
CML
Myelofibrosis
A pt presents w/ pruritis, fatigue, HA and upon examination has ruddy complexion and mild spenomegaly. What is the likely diagnosis and what mutation is associated with this?
Polycythemia vera
–> JAK 2 mutation
How would you go about treating a pt w/ polycythemia vera?
Phlebotomy until they are Fe deficient
Tx associated symptoms (gout, pruritis, HA/Vertigo)
A pt w/ polycythemia vera has an increased Hct. What is the diagnostic Hct and what is seen more commonly in males v. females?
Dx: >60% Hct
Usually see levels however of:
Males = 52-60%
Females = 48-60%
What are the differential diagnosis for Polycythemia vera? (3)
- Gaisbock’s Syndrome
- Chronic Hypoxia
- Renal Cell CA
Treatment for essential thrombocytosis if asymptomatic
Observation w/ routine follow up and pt education on worrisome symptoms
Treatment for essential thrombocytosis if symptomatic or above a certain platelet count (which is…)?
Platelets > 1 million warrents tx
1st line = either anagrelide or hydroxyurea to lower platelet count
____ is almost diagnostic of CML but the sine qua non of CML is _____.
Basophilia
Sine qua non- Philadelphia chromosome
Which s/s of CML are worryisome and require urgent treatment?
Thrombocytosis of > 1million associated w/ wet purpura, GI bleeding, or thrombosis
2 phases of CML
Chronic Phase
Accelerated Phase
Blast Crisis
What is the treatment goal of CML and what does this mean?
Goal is complete molecular remission meaning no evidence of BCR-ABL1 transcripts by RT-PCR
The tyrosine kinase inhibitor used to tx CML is called ______.
Imatinib (Gleevec)
Leuko-erythroblasts (immature WBC and RBC that are tear dropped shaped) are indicative of what disease?
Myelofibrosis Myeloid Metaplasia (MMM)
Poor prognosis factors of Myelofibrosis myeloid metaplasia (5) and what the risk/average survival rate that correlates with these risks
Risk:
- Age > 65yo
- Constitutional Sx
- Hgb 10%
- WBC > 25,000
Low Risk = 0 of these sx = 11year survival
Intermediate 1 = 1 sx = 8 yr survival
Intermediate 2 = 2 sx = 4 yr survival
High Risk = 3+ sx = 2 yr survival
How are GI cancers staged?
TNM System (Tumors, Nodes, Mets)
Using the TNM staging system for colon CA: what is stage 0, 1A, 1B, 2, 3A, 3B, 4
Stage 0: Tis N0 Stage 1A: T1, N0 Stage 1B: T2, N0 Stage 2: T1,T2 N1 or T3 N0 Stage 3A: T3 N1,2 Stage 3B: T4 Stage 4: Mets
Most GI CA arises from _____ and the most common type is ______.
Arise from epithelial layer of mucosa
MC type = adenocarcinoma
Upper and mid esophageal CAs are mostly _________ (type)
Squamous Cell
Tx for Tis or high grade dysplasia or T1 =
Endoscopic ablation
Esophageal resection
Although gastric cancer has declined world-wide, where in the world is it still a common problem?
Japan and some other areas of Asia
Factors contributing to gastric CA
- Diets low in vitamins A&C
- Consumptin of smoked or cured foods
- Untreated infection w/ H. pylori
- Genetic Predispositions (Blood type A, Pernicious anemia)
Most gastric CA are _______ (type)
Adenocarcinomas
The 3rd most common and 3rd most lethal CA in the US is _____.
Colorectal CA
Primary Prevention of GI Cancers: (4 things)
- High fiber and low fat diets
- NSAIDs
- Ca++
- HPV Vaccine (prevent anal CA)
Colorectal CA screening
Start @ 50yo or 10 years before age that 1st degree relative was dx’d w/ CRC
How often does fecal blood occult test miss colon cancer?
50% of the time (false negative)
How often does flixble sigmoidoscopy miss colon CA?
50% of the time
What drug is the backbone of Rx for almost all GI cancers?
5FU (5 Fluorouracil)
Do GISTs respond well to chemo?
NO!
Are soft tissue sarcomas cancers of children or adults?
Adults
Most common CNS cancer is ______.
Metastatic (lung, breast, pancreas, melanoma)
Most common bone cancer is _____.
Metastatic (prostate, breast, lung)
Sickle cell deforms RBCs causing _______, ________, and _______.
Hemolysis
Splenic sequestration
Microvascular occlusion
Splenic infarction causes overwhelming infection by encapsulated organisms. These organisms are?
Strep
Hemophilus
Klebsiella
Salmonella
How to dx sickle cell anemia?
Hg Electrophoresis –> detects HbS (trait has 40% and disease has >95%)
Blood smear –> sickled cells, hemolysis, reticulocytosis, nucleated RBCs, target cells, hollow jolly bodies d/t autosplenectomy
Tx of sickle cell anemia. How does this tx work?
Hydroxyurea
- Increases HbF which decreases HbS and painful crisis
- Also decreases WBCs and retics (could worsen sx)
Warm autoimmune hemolytic anemia results in _______ hemolysis whereas cold autoimmune hemolytic anemia results in ______ hemolysis.
Warm = IgG = extravascular hemolysis
Cold = IgM = intravascular hemolysis
Most common cause of B12 deficiency
Pernicious Anemia
–Lack of intrinsic factor
How do you distinguish B12 and Folate deficiency?
B12 has increased homocysteine levels and increased MMA levels whereas Folate has increased homocysteine but NORMAL MMA levels.
B12 also involves peripheral neuropathy whereas folate does not
Causes of Folate Deficiency Anemia
- Inadequate intake (alcoholics)
- Increased requirement (pregnancy)
- Defective absorption (celiacs)
- Exposure to folic acid antagonists (methotrexate, trimethoprim, anticonvulsants)
What 3 drugs are folic acid antagonists?
- Methotrexate
- Phenytoin
- Trimethoprim
EPO is secreted by the ______ and ___ is its main stimulus
Kidney
02
What are 3 substrates needed for EPO to create new RBC
Folate
B12
Iron
Life cycle length of RBC, platelet, WBC
RBC = 120 days Platelet = 7 days WBC = 1 day
Which two populations usually have low RBC mass
Elderly
African Americans
What are 4 instances in which there are changes in plasma volume that may decrease or increase RBC concentration and skew lab results
- Acute Bleeding (dec. volume may show nml Hgb and Hct levels)
- When nml volume is restored (pt might demonstrate anemia)
- Dehydration (when dydrated the Hgb & Hct fall)
- Pregnancy (plasma volume increases faster than RBC therefore pt may appear falsly anemic)
In a pregnant women the plasma concentration increase by ____% whereas the RBC increase by ___%.
Plasma inc. 50%
RBC inc. 25%
Heinz Bodies =
Thalassemia!
Complications of Beta-Thalassemia Major (Cooley’s Anemia)
- Growth retardation
- Severe anemia
- Abnormal facial structures
- Fx & Osteopenia
- Hepatosplenomegaly
- Jaundice and bili gall stones
- High output CHF
- Short life span (<30yo)
Target cells, Basophilic stippling, heinz bodies =
Beta thalassemia
Transfusion complications of thalassemia:
Iron Overload –> heart failure, liver failure, pancreatic failure, skin pigmentation
A 70 yo pt presents with fatigue, SOB, and angina. You order a CBC and results come back noting anemia, thrombocytopenia, and leukemia. Upon peripheral smear the lab techs notice ringed sideroblasts and increased number of blast cells. What is the diangosis? If a pt was older and had a high level of blasts (~15%) what would be your suspected treatment?
Myelodysplastic Syndrome
If a pt had increased blasts their average survival rate decreases and especially if the pt was older I would choose supportive tx and not put on chemo since this cancer is not curable
Gingival hyperplasia is a tip off for what type of leukemia?
Monocytic Leukemia
Which is more common: non-hodgkins lymphoma or Hodgkin’s Lymphoma?
NHL
Are NHL mostly B cells or T cells
85% B cell
The 2nd most common of all adult lymphomas is _______ which accounts for ____%
Follicular Lymphomas
30%
Most non-hodgkins lymphomas, Waldenstrom’s Macroglobulinemia, and Multiple Myeloma are diseases of ___ cells
B cells
MALT Lymphomas stand for what?
Mucosally Associated Lymphoid Tissue
