Heme - Onc Flashcards
Extramedullary Hematopoiesis is most commonly caused by what?
[Beta-thalassemia] = [Chronic Hemolytic Anemia]
Explain the 2 steps of the Schilling Test
1st: [Oral Vit B12] is given with [IV Vit B12] –> Excess [Vit B 12]. Urine is collected and should show [HIGH urinary excretion] of [Vit B12]= indicates [Vit B12] was [intestinally absorbed] properly into the circulatory system
(low urinary excretion = possible malabsorption problem and rules out dietary deficiency)
2nd: Repeat, but do with [Intrinsic Factor]
(If it corrects = IF deficiency was the issue)
( If doesn’t correct = TRUE MALABSORPTION PROBLEM)
Name 4 Common causes of Vitamin B12 Deficiency
- GIP [Gastritis / Ilial Resection / Pancreatitis]
- Fish Tapeworm
- Atrophic Gastritis –> Destruction of [Parietal Cells that produce IF] –> DEC [Intrinsic Factor]
- Strict Vegetarians (Proteins contain Vit B12)
A: List the Dx Criteria for SLE - Systemic Lupus Erythematosus
B: How many of these criteria must be met for diagnosis?
BUBA’S a KAPPA Dude
- Butterfly Malor Rash
- Ulcers (Oral vs. Nasal)
- Brain Dz (seizures / psychosis)
- Arthritis (can tx w/anti-malarial)
- Serositis (pericarditis / pleurisy / peritonitis)
- Kidney Dz (Lupus Membranous Nephropathy) vs. (Lupus Nephritis)
- Anti-nuclear Antibodies
- Pancytopenia (includes compliment)
- Photosensitivity
- Autoantibodies (dsDNA vs. Smith vs. Phospholipid(False positive VLDR) )
- Discoid Rash anywhere on body
B: At least 4
Where does Extramedullary Hematopoiesis for this condition occur? (2)
Liver and Spleen
Classic presentation (3) for [Peau d’Orange (inflammatory Breast CA)]
and MOD
[Erythematous / itchy / indurated (orange peel) Rash] caused by
CA spreading to dermal lymphatic space –> obstructing lymphatic drainage
Key Features of [Ductal Carcinoma In Situ] (3)
Invasive or NonInvasive?
Key Features of [Paget Breast CA] (2)
Invasive or NonInvasive?
Key Features of [Ductal Carcinoma] (2)
Invasive or NonInvasive?
Key Features of [Lobular Carcinoma] (2)
Invasive or NonInvasive?
The HER2Neu oncogene encodes for a ______ glycoprotein that has intracellular _____ activity and is in the ____ family. This ultimately does what?
The HER2Neu oncogene encodes for a [185kD transmembrane] glycoprotein that has intracellular Tyrosine Kinase activity and is in the EGFR family. Ultimately accelerates cell proliferation.
How does Warfarin cause Skin Necrosis? Which demographic? Onset?
Warfarin inhibits Protein C and S (natural anticoagulants) –> Skin Necrosis.
People deficient in these are at INC risk
Onset = first few days of warfarin therapy
Manifestations of Atrophic Gastritis (3)
- hypOchlorhydria
- DEC IF production –> VitB12 Deficiency
- INC methylmalonic acid
[Identify and Describe Arrows] and then Dz
AML (Acute Myeloid Leukemia)
[Blast = immature myeloid precursors that replace normal bone marrow]–> Pancytopenia
Auer rods = [Peroxidase linear puple inclusions]
Von Willebrand Dz
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
[IITP - Immune Idiopathic Thrombocytopenic Purpura]
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
TTP-HUS
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
DIC
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
[IITP - Immune Idiopathic Thrombocytopenic Purpura]
MOD
Autoantibodies destroy platelets –> Isolated Thrombocytopenia w/ NO splenomegaly
Clinical Sx for [Systemic Lupus Erythematosus] (8)
- Constitutional (Fever / Fatigue / Wt. Loss)
- Arthritis -Symmetric & Migratory
- [Malor Butterfly Rash]
- Photosensitivity
- Serositits (pericarditis & peritonitits)
- Thromboembolism (due to antiphospholipid Ab)
- Seizures & Cognition DEC
- [Lupus Nephritis = PrOteinuria + INC Creatinine (Type 3) Hypersensitivity]
Labs for [Systemic Lupus Erythematosus] (4)
- Pancytopenia (Hemolytic Anemia Type 2 Hypersensitivity) / Thrombocytopenia / Leukopenia)
- hypOcomplent (C3 & C4)
- [ANA Ab (sensitive)]
- [Anti-dsDNA and SM (specific)]
Anaplastic tumors are ____ and bear no resemblance to the original tissue. They often contain _____ cells and _____
Anaplastic tumors are Undifferentiated and bear no resemblance to the orginial tissue. They often contain [Giant Multinucleated Tumor cells] and Pleomorphism
Name a common and serious complication of [Chronic Hemolytic Anemia] with Blood transfusion.
How is this treated?
Hemosiderosis (Iron overload) - typically manifested as Hemosiderin accumulation in Kupffer macrophages.
Tx = Iron Chelation
Name the CA that cause [OsteoBLastic Bone Metastases] (3)
Prostate
SOLC
Hodgkin Lymphoma
Name the CA that cause BOTH [OsteoBLastic and Osteolytic Bone Metastases] (2)
GI
Breast
Name the CA that cause [Osteolytic Bone Metastases] (5)
Vitamin B12 and Folid acid deficiency present similarly. What is their difference and why?
Neuro dysfunction is only seen in VitB12 deficiency;
B12 deficiency –> axonal demyelination in [peripheral n. / spinal cord (Posterior vs. Lateral column) / cerebrum]
[G6PD -Glucose 6 phosphate Dehydrogenase] deficiency
Mode of Inheritance
X-Linked recessive
Hereditary Spherocytosis
Mode of Inheritance
Auto Dom
Pts with [G6PD deficiency] are at risk for _____ when taking _____ before going to Africa. Peripheral blood smear will show _____
medication induced oxidative stress; Antimalarials; Heinz Bodies (dark RBC inclusions that stain with supravital stain)
Laboratory manifestation of [G6PD deficiency] (4)
intravascular and extravascular hemolysis with
anemia
reticulocytosis
[UIB - Unconjugated Indirect Bilirubinemia]
[low haptoglobin from DEC in HgB-Haptoglobin complexes]
Dactylitis (painful swelling of ______) is a common presentation of _____ in ______
Dactylitits (painful swelling of Hands and Feet) is a common presentation of Sickle Cell in Kids
[Sickle Cell Anemia] Labs (3)
INC [UIB - Unconjugated Indirect Bilirubin]
INC Lactate Dehydrogenase
DEC Haptoglobin
[Sickle Cell Anemia]
Mode of Inheritance
auto recessive
Most common auto recessive DO in Blacks
List and Describe the 3 main pathogenic features of [Sickle Cell Anemia]
- Hemolysis: RBC Sickling–>Deformed RBC destruction by macrophages & mechanical stress = intra/EXtravascular hemolysis–> Jaundice/Sclera Icterus. Haptoglobin binds to INC free HgB preventing Renal injury
- Vasoocclusion: Hypoxic tissue injury from occlussion–>Pain Crisis, Dactylitis (infarct in joint bones), Priapism and Autosplenectomy
- Infection: Autosplenectomy –> inability to fight encapsulated bacteria –> [Staph & Salmonella Osteomyelitis]
Name the [Vitamin K-dependent Coagulation factors] (4), where they’re made and why they’re VitK-dependent
Factors 2/7/9/10; made in Liver
7 has shortest half life
These are initially made in liver and then activated by [VitK-dependent carboxylation]
Failure of ProThrombin Time to correct with VitK supplementation indicates _______
[Factor 7 deficiency from Liver Dz (Cirrhosis)]
Dermatomyositis Clinical Presentation (4)
Autoimmune Dz –>
- [Proximal m. weakness (resembles polymyositis)]
- [PeriOrbital Heliotrope Rash]
- Gottron’s Papules - in image
- [Mononuclear Perimysial infiltrates on histo]
Which CA is Dermatomyositis associated with (4)
- Ovarian
- Colorectal
- Lung
- Non-Hodgkin Lymphoma
Gottron’s Papules in image
Clinical Presentation for [Hairy Cell Leukemia] (3)
B-Cell neoplasm –>
- Bone Marrow Failure –> [Splenomegaly from reticuloendothelial infiltration]
- Unsuccessful Bone marrow aspiration (Dry Tap)
- Lymphocytes with cytoplasmic projections-image
Demographic for [Hairy Cell Leukemia], and how is it diagnosed?
[Middle Aged men]
[TRAP - Tartrate Resistant Acid Phosphatase] Flow cytometry
[Factor 5 Leiden] MOD (2)
- Factor 5 is genetically altered to resist protelysis/inactivation by [Activated Protein C] –> [INC DVT/PE and Pregnancy Lost]
- Factor 5 Leiden can not support APC’s anticoagulant activity –> [INC DVT/PE and Pregnancy Lost]
What’s the most common cause of [Inherited Thrombophilia]?
Factor 5 Leiden
Labs for CML - Chronic Mylogenous Leukemia (2)
- [Elevated Myelocyte WBC]
- [DECREASED (Leukocyte alkaline phosphatase)] - differentiates CML from Leukemoid rxn
CML genetic cause (2)
PDGF mutations caused by:
Philadelphia Chromosome (translocation between Chromo 9 & 22)
vs.
BCR-ABL1 fusion gene
BCL2 function
Inhibits Apoptosis –> promotes cell survival –> (Follicular) Lymphoma
BCL2 oncogene is associated with ______. What’s the MOD
BCL2 oncogene is associated with Follicular Lymphoma
Translocation of [BCL2 oncogene] from Chromo 18–>[Chromo 14 IgHeavy Chain] –> BCL2 Overexpression –> inhibits too much apoptosis
[SPEP - Serum Protein ElectroPhoresis] showing elevated [gamma-globulin] usually represents a ___ consisting of overproduced _____. What Dz is this?
SPEP showing elevated [gamma-globulin] usually represents a M protein consisting of overproduced [monoclonal immunoglobulin] = MULTIPLE MYELOMA
Multiple Myeloma Clinical Presentation (6)
Crazy Ass BUMP
- [AL amyloidosis]-from monoclonal (Ig light chains)
- Bone marrow w/plasmacytosis - shown in image
- Urine IgG
- [Mott cells on Histo]
- Proteins (M Protein) in serum
Triad Presentation for [PNH - Paroxysmal Nocturnal Hemoglobinuria]
PNH
- [Hemolytic Anemia –> Hemoglobinuria & Potential Renal Hemosiderosis (iron deposit)]
- [Pancytopenia from stem cell damage]
- [Novel (atypical) Thrombosis locations (mesenteric vein)]
[Paroxsymal Nocturnal Hemoglobinuria] Cause and how it affects complement
[Complement-mediated hemolysis] caused by a [mutated PIGA gene] which normally makes [GPI anchor protein].
[GPI anchor protein] anchors markers like [CD55 Decay accelerating factor] and [CD59 MAC inhibitory protein] which both inactivate complement
How do [ProInflammatory IL1 & IF-gamma] affect VEGF
Both INC VEGF expression –> promotes angiogenesis
[FGF2 - Fibroblast Growth Factor] Function (4)
- Angiogenesis
- Embryonic Development
- Hematopoiesis
- Wound Repair (recruits macrophages/fibroblast/endothelial cells)
What are [Schistocyte Helmet cells] and what are they caused by (2)
Fragmented RBC caused by
- [HUS HAT microangiopathic hemolytic anemia]
vs.
- [Prosthetic Cardiac Valves] - mechanical damage
* Both –> Intravascular Hemolytic Anemia*
[Intravascular Hemolytic Anemia] Labs (3)
- DEC Haptoglobin (due to Hptoglbn binding to INC free Hgb)
- INC LDH
- INC [Unconjugated indirect Bilirubin]
Aplastic Anemia MOD
[Hematopoietic Stem Cell Deficiency (CD34)] –> Bone Marrow Failure
Aplastic Anemia Causes (4)
Aplastic Anemia Clinical Presentation (4)
- NO Splenomegaly
- Anemia
- Thrombocytopenia
- Leukopenia
How do you diagnose Aplastic Anemia
Polycythemia Vera MOD
Uncontrolled RBC production due to [JAK2 mutation]
Polycythemia Vera Clinical Presentation (6)
- Aquagenic Pruritus
- Facial Plethora
- Splenomegaly
- Gouty Arthritis
- RBC Mass INC - Lab
- [Thrombocytosis & leukocytosis] - Lab
[TTP-HUS] MOD
[DEC ADAM-TS13 protease] –> [Uncleaved Large vWF multimers] –> Diffuse [microvascular platelet thrombi] –> [HUS HAT] and thrombocytopenia
[TTP-HUS] Clinical Presentation (4)
- [Hemolytic Anemia with (Schistocyte Helmet Cells)]
- Thrombocytopenia
- Renal Failure
- Neurologic Sx
Identify the Cell and what Dz it’s associated with
[RADC - Reactive Atypical Downey CD8] Cell -Controls EBV infection
[____ Deficiency] commonly occurs with Alcoholism and –> ____ anemia from _____
[Folic Acid Deficiency] commonly occurs with Alcoholism and –> Megaloblastic Anemia from impaired DNA base synthesis
VitB12 can also be deficient
Folic Acid and [VitB12/Cobalamin] deficiencies are both associated with Alcohol. How can you differentiate?
Folic Acid Deficiency 2° to EtOH = Acute usage (onset within weeks)
[VitB12/Cobalamin] Deficiency 2° to EtOH = Chronic usage
MOA for how [RetinoBlastoma gene] contributes to CA
Proliferation signals activate CDK4–> [Hyperphosphorylates Rb protein] –> prevents Rb from binding to [E2F tx factor].
[E2F tx factor] promotes cells to go from G1–> S –> [Division by DNA polymerase]!
[G0 resting cells] contain active hypOphosphorylated Rb
Von Willebrand Dz
MOD
Qualitative defect in [platelet binding & aggregation] –> [Lifelong INC Bleeding time from Skin & Mucosal(menorrhagia vs. epistaxis)]
Pts have normal Platelet Count
How does [Von Willebrand Factor] normally function (2)
Promotes Platelet Adhesion by binding [Gp1B Platelet Glycoproteins] to [exposed Subendothelial Collagen]
AND
Acts as protective carrier protein for Factor 8 (INC its half life)
Why is HgB A<strong>2</strong> elevated in beta-thalassemia?
[Beta-globin chain] underproduction –> DEC HgB A<strong>1</strong> synthesis–> elevated HgB A<strong>2</strong> to compensate
Remember that HgBA1C is used to measure DM glycemic control
How does TNF-a (AKA ____) affect Metabolism (2). What cell secretes it?
- Suppresses appetite
- INC BMR
- TNF-A (AKA Cachectin) –> paraneoplastic Cachexia*
- Secreted by macrophages*
Describe the following levels for [Beta Thalassemia minor]
A: HgB A1
B: HgB A2
C: HgB Fetal
D: HgB S
Describe the following levels for [Beta Thalassemia MAJOR]
A: HgB A1
B: HgB A2
C: HgB Fetal
D: HgB S
Describe the following levels for [Sickle Cell trait]
A: HgB A1
B: HgB A2
C: HgB Fetal
D: HgB S
Describe the following levels for [Sickle Cell DZ]
A: HgB A1
B: HgB A2
C: HgB Fetal
D: HgB S
[Von Willebrand Dz] Mode of Inheritance
AUTO DOM + variable penetrance