-
Describe the Intrinsic Coagulation Pathway
Contact Activation Pathway
High Molecular Weight Kininogen + Factor XII starts cascade with collagen and prokallikrein
-
Describe the Extrinsic Coagulation Pathway
This is called the Tissue Factor Pathway
Thromboplastin (aka Tissue Factor) activates Factor VII and X.
-
What is the rate limiting factor in the clotting cascade?
Factor X
-
What are the two accelerating factors in the clotting cascade?
Factor V and VIII
-
What two proteins inhibit the accelerating factors in the clotting cascade?
Protein C and S (thrombotic)
-
Describe the role of HMWK in the coagulation cascade.
It activates bradykinin which stimulates the kinin pathway:
increased vasodilation, permeability and pain
-
Hemophilia A is a defect in what factor? Hemophilia B?
- A - 8 (a-ate)
- B - 9 (benign)
-
Vitamin K deficiency results in the decreased synthesis of what factors?
DISCO 1972
Factors 2, 7, 9 and 10, Proteins C and S
-
Antithrombin (an anticoagulant) inhbits what factors? What drug activates antithrombin?
Inhibits: thrombin (increases clotting), 2,7,9,10,11 and 12
Activated by heparin
-
What enzyme activates Vitamin K to allow creation of coagulation cascade components? What drug inhibits this enzyme?
Epoxide reductase
Warfarin
-
What counter-intuitive side effect can be seen with Warfarin?
Transient hypercoagulation at first administration.
Proteins C and S are inhibited, but they usually stop accelerating factors V and VIII, so they are temporarily unchecked.
-
tPA is used clinically as a thrombolytic. How does it work?
Plasminogen is turned into plasmin by tPA (tissue plasminogen activator) which cleaves fibrin mesh.
-
What does PT test for? What does PTT test for?
PT - tests extrinsic pathway.
PTT - tests intrinsic pathway
-
In Hemophilia A or B, what test, PT or PTT, would be increased?
PTT - checking for factors 8 and 9 which are found only in intrinsic pathway.
-
Which tests are elevated in Vitamin K deficiency? PT or PTT?
Both - DISCO1972 describes factors in both pathways.
-
Babies get a shot of this vitamin in the hospital because gut flora haven't yet developed to produce it.
Vitamin K
-
If you have a disorder of the platelets only, what test would you expect to see elevated?
Coagulation factors are fine, so you only get an increase in bleeding time (BT) and PT, PTT are normal.
-
Factor V Leiden disease - Pathogenesis and Symptoms
Mutated Factor V = super fast clotting
Hypercoagulability
-
What is the most common cause of hereditary hypercoagulability?
Factor V Leiden
-
What do you see with a prothrombin gene mutation?
venous clots
-
What do you see with an antithrombin deficiency?
resistance to heparin (which activates antithrombin, so if there isn't any in the blood - can't do its work)
Hypercoag
-
What do you see in a Protein C or S deficiency?
decreased ability to inactivate factors V and VIII - hypercoag. increased risk of thrombotic skin necrosis after administration of Warfarin - can't shut off clotting cascade!
-
Heparin - MOA, Clin Use, Toxicity
MOA: activates antithrombin
Clin Use: Pulmonary Embolism, Stroke, Acute Coronary Syndrome, MI, DVT - immediate anticoagulation
Toxicity: HIT, Bleeding, Osteoporosis
-
This anticoagulant is safe for pregnant women.
Heparin
-
LMW Heparin: MOA, Clin Use, Tox:
MOA: acts on Xa instead of antithrombin only. Longer T1/2
Clin Use: subcutaneous administration, less monitoring
Tox: Not easily reversible
-
What drug can you give to reverse too much Heparin?
protamine sulfate
-
Heparin Induced Thrombocytopenia - - Pathogenesis and Symptoms
Heparin binds Factor 4 - this starts immune response and attacks platelets.
Thrombocytopenia and hypercoagulable state
-
What drug do you give to someone with HIT instead of Heparin? What is its MOA?
Lepirudin - inhibits thrombin directly
-
Warfarin - MOA, Clin Use, Tox
MOA: inhibits epoxide reductase / Vitamin K
Clin Use: Chronic anticoagulation (DVT prophylaxis), A Fib
Tox: Teratogenic, Bleeding, Necrosis,
-
What values should you test regularly when your patient is on Warfarin?
INR and PT
-
Compare Warfarin to Heparin: Structure, Route of Administration, Site of Action, Onset of Action, MOA, Treatment of Overdose, Monitoring and Placental cross
- Structure: H - large, W - small
- ROA: H - IV/SC, W - oral
- SOA: H - blood, W - liver
- OOA: H - rapid, W - slow (limited by factor T1/2's)
- MOA: H - activates antithrombin, W - inhibits epoxide reductase
- OD Tx: H - Protamine Sulfate, W - IV Vit K and Fresh Frozen Plasma
- Monitor: H - PTT, W - PT/INR
- Placental Cross: H - No (safe for preg), W - yes (teratogenic)
-
Name 4 thrombolytic agents and their MOA:
- Streptokinase
- Urokinase
- tPA (alteplase)
- APSAC (anistreplase)
MOA: converts plasminogen to plasmin - cleaves thrombin or fibrin clots.
-
What do you see in terms of tests when tPA is administered?
Increased PTT and PT
-
What is the clinical use for thrombolytics?
Early MI, Early Stroke
-
What can you use to reverse bleeding from thrombolytics?
Aminocaproic Acid
-
Does enoxaparin (LMWH) need to be monitored?
No
-
What is the treatment for heparin induced thrombocytopenia?
Hirudin (Lepirudin) drugs and protamine sulfate
-
Where does fetal erythropoiesis occur?
- Yolk Sac - 8weeks
- Liver - 30 weeks
- Spleen - 28 weeks
- Bone marrow - >28 weeks
-
How does fetal hemoglobin differ from adult hemoglobin?
- Fetal - 2 a chains, 2 y chains - higher affinity for O2
- Adult - 2a, 2b chains
-
Erythrocyte: shape, energy source, survival time
-
What is the protein responsible for the flexibility of RBC's?
Spectrin
-
What word describes RBC's of:
1) varying size
2) varying shape
3) immature
- Anisocytosis - size
- Poikilocytosis - shape
- Reticulocyte - immature
-
What kind of hypersensitivity reaction happens if you transfuse the wrong blood type?
Type II
-
What blood type is considered the universal donor? Universal recipient?
-
Describe the 4 steps of platelet plug formation
- 1. Injury - vWF binds collagen
- 2. Adhesion - platelets bind vWF and release ADP and Ca2+
- 3. Activation - ADP induces GpIIb/IIIa expression on platelet
- 4. Aggregation - Fibrinogen binds GpIIb/IIIa and links platelets.
-

Cell Type and Associated Path:
Acanthocyte (spur cell)
- Liver Disease
- Abetalipoproteinemia
-
Cell Type and Associated Path:
Basophillic Stippling
"Baste the Ox TAIL"
- Thalassemia
- Anemia
- Iron Deficiency
- Lead Poisoning
-
Cell Type and Associated Path:
Bite Cell
- G6PD deficiency
- -> can't go through HMP shunt, causing oxidative damage resulting in Heinz Bodies which are chewed on by macrophages in the spleen
-
Cell Type and Associated Path:
Macroovalocyte
Megaloblastic Anemia
-
Cell Type and Associated Path:
Ringed Sideroblast
- Sideoblastic Anemia
- Lead Poisoning
-
Cell Type and Associated Path:
Schistocyte - Helmet Cell
DIC, TTP/HUS, Traumatic Hemolysis
cells are forced through fibrin areas and get chopped up
-
Cell Type and Associated Path:
Sickle Cell
Sickle Cell Anemia
-
Cell Type and Associated Path:
Spherocyte
Hereditary Spherocytosis, Autoimmune Hemolysis
-
Cell Type and Associated Path:
Teardrop cell
Myelofibrosis
-
Cell Type and Associated Path:
Target Cell
HALT!
- HbC disease
- Asplenia
- Liver Disease
- Thalassemia
-
Cell Type and Associated Path:
Heinz Bodies
iron oxidation
seen in : a-thalassemia, G6PD deficiency
-
Cell Type and Associated Path:
Howell-Jolly Body
Functional Apslenia - Sickle cell, splenic trauma, TTP/HUS
-
Name the 3 different types of Polycythemia
- Appropriate Absolute
- Inappropriate Absolute
- Polycythemia Vera
-
Which type of polycythemia is associated with Lung disease, high altitude and congenital heart disease?
appropriate absolute - incresaed RBC mass decreased O2 saturation
-
Which type of polycythemia is associated with RCC, HCC, Wilms tumor and hydronephrosis?
inappropriate absolute - due to neoplastic ectopic erythropoietin
-
What is the pathogenesis of polycythemia vera?
Monocolonal proliferation of RBC's
-
Lead Poisoning: Affected enzyme and presenting symptoms and treatment
Ferrochelatase and ALA dehydratase
microcytic anemia, GI and kidney disease
- Kids: mental deterioration
- Adults: headache, memory loss
Tx: Dimercaperol, EDTA, Succinyl
-
Acute Intermittent Porphyria: Affected enzyme, presenting symptoms and treatment
Porphobilinogen deaminase
- Acute abdomen, acute neuro pain
- Dark red wine urine, psych disturbances
Tx: glucose and heme
-
Porphyria Cutanea Tarda: Affected enzyme and presenting symptoms
Uroporphyrinogen decarboxylase
Homeless Guy!
skin blister, photosensitivity, tea colored urine, excessive hair, related to alcohol, Hep C
-
Which is the most common porphyria?
Porphyria cutanea tarda
-
What is the rate limiting step in heme synthesis?
aminolevulinic acid synthase
-
Name the 9 different types of Hemoglobin
- HbA
- HbA2
- HbA1c
- HbF
- HbGower
- HbS
- HbC
- HbBarts
- HbH
-
Which hemoglobin is the most common?
HbA
-
Which hemoglobins are associated with severe a-thalassemia?
HbBarts and HbH
-
Which hemoglobin is associated with fetal hb? How is it different?
HbF
two gamma chains instead of beta chains (in adults)
-
What is substituted in HbS in Sickle Cell?
Valine in B chain
-
What is substituted in HbC in HbC Disease?
Lysine in B chain
-
Name the 5 microcytic anemias
TAILS again, like in Basophillic stippling
- Iron deficiency
- ACD
- Thalassemias
- Lead Poisoning
- Sideroblastic Anemia
-
Iron Deficiency Anemia: Disorder and Findings
Associated Disease?
Chronic bleeding, malnutrition, decreased heme synthesis
Microcytosis and hypochromia
Plummer Vinson syndrome (DIG - Dysphagia, Iron Deficiency, Glossitis)
-
A-thalassemia: Disorder and Findings
defect in a-globulin chain
Africans and Asians
- 4 gene deletion - HbBarts = hydrops fetalis
- 3 gene deletion - HbH disease
-
B-thalassemia: Disorder and Findings
point mutation -> decreased B globin
Mediterranean people
- Minor: asymptomatic
- Major: chipmunk facies, crew cut on skull. Severe anemia, requires transfusions
Major blood smear findings: weird shapes and sizes of RBC's, target cells, helmet cells, hypochromia
-
Lead Poisoning: Disorder and Findings
Lead inhibits ferrochelatase and ALA dehydratase
- "LEAD BullShit"
- Lead lines on long bones and gingiva
- Encephalopathy
- Abdominal Colic
- Drops - foot, wrist
- Basophillic stippling
- Sideroblastic Anemia
Tx: Dimercaprol, EDTA, Succimer
-
Where are ringed sideroblasts found?
In the bone marrow
-
When is ACD seen and how is it different from iron deficiency?
ACD - osteomyelitis, malignancy
It's NOT iron deficiency because the TIBC is low saying "we have iron but we can't use it"
-
In Iron deficiency anemia, is the TIBC high or low?
TIBC is high - it's ready for iron!
-
Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Iron Deficiency
- SI: low
- TIBC: high
- Ferritin: low
- % Sat: Super Low
-
Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: ACD
- SI: low
- TIBC: low (can't use it)
- Ferritin: high (we've got it stored)
- % Sat: -
-
Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Hemochromatosis
- SI: high
- TIBC: low
- Ferritin: high
- % sat: high
Shitloads of iron!!
-
Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Lead Poisoning
- SI: high
- TIBC: low
- Ferritin: -
- % sat: high
-
What should you rule out in a male over 50 with new onset iron deficiency anemia?
colon cancer
-
What test can be used to diagnose B-thalassemia minor?
HbA2 will be elevated
-
Folate Deficiency: Disorder and Findings
Megaloblastic Anemia
alcoholism, malnutrition, drugs (methotrexate), pregnancy
Hypersegmented neutrophils! Glossitis, increased homocysteine
-
B12 deficiency: Disorder and Findings
Megaloblastic Anemia
Vegans, pernicious anemia, fish tapeworm
Hypersegmented neutrophils - neurological symptoms! increased homocysteine
-
If a patient has megaloblastic anemia but they are not folate or B12 deficient, what is the disease?
Orotic Aciduria
-
What are some causes of nonmegaloblastic macrocyctic anemias?
- Liver disease - alcoholism
- orotic aciduria
- congenital deficiencies of purine/pyrimadine synthesis, drugs which increase erythropoietin
-
Anemia of Chronic Disease (ACD): Disorder and Findings
Increased inflammation causes increased hepcidin from liver which prevents release of iron from macrophages
can go to microcytic hypochromic
-
Aplastic Anemia: Disorder and Findings
Nonhemolytic normocytic anemia
- Due to destruction of myeloid stem cells:
- radiation, drugs, viral agents, Fanconi's anemia (DNA repair defect)
Bone marrow gives up - see lots of fat, few cells.
Sx: fatigue, malaise, bleeding, petechiae, purpura, infection
Tx: marrow transplant
-
What viruses can cause aplastic anemia?
-
Intravascular Hemolysis: Pathogenesis and Findings
decreased haptoglobin, increased LDH and hemoglobin in urine
Paroxysmal nocturnal hemoglobinuria, mechanical destruction due to prosthetic valve
-
Extravascular Hemolysis: Pathogenesis and Findings
Macrophages in the spleen clear the RBCs - increased LDH and unconjugated bilirubin -> jaundice. Hereditary spherocytosis, G6PD deficiency, sickle cell
-
Hereditary spherocytosis: Description, Findings and Tx:
Intrinsic Hemolytic Normocytic Anemia
defect in ankyrin and spectrin
anemia, jaundice, pigmented gallstones, splenomegaly, aplastic crisis (Parvo B19)
Howell Jolly bodies and increased osmotic fragility test
Tx: Splenectomy
-
Coombs Tests: Direct vs. Indirect
Direct - antibody finds antibody - used in hemolytic anemias
Indirect - patient serum finds antibodies to foreign blood
-
Cold Agglutinins vs. Warm Agglutinins
- Cold: IgM - MMM Cold Ice Cream
- low temp agglutination
- Mycoplasma Pneumonia, EBV, Malignancy, CLL
- Warm: IgG Warm is GGGreat!
- Viruses (EBV, HIV)
- SLE
- Malignancies (CLL), nonHodgkin Lymphoma
- Congential Abnormalities
-
G6PD Deficiency: Description/Defect, Findings and Tx:
Intrinsic Hemolytic Normocytic Anemia
x linked
- RBC increased oxidative stress
- sulfa drugs, infections and fava beans
- Heinz Bodies and Bite Cells
Back Pain then Peeing Blood
-
Pyruvate kinase deficiency: Description/Defect, Findings and Tx:
Intrinsic Hemolytic Normocytic Anemia
Decreased ATP
Hemolytic anemia of newborn
-
Paroxysmal Nocturnal Hemoglobinuria: Defect, Findings and Tx
Intrinsic Hemolytic Normocytic Anemia
complement mediated RBC lysis
CD55 and CD59 missing, Hams test
Labs show increased hemosiderin in urine
-
Sickle Cell Anemia: Defect, Findings, Tx
Intrinsic Hemolytic Normocytic Anemia
HbS point mutation, low O2 or dehydration causes sickling
Crew cut on skull (increased erythropoiesis), aplastic crisis, autosplenectomy (increased risk of infection with encapsulated organisms), Howell-Jolly bodies, Salmonella osteomyelitis, renal papillary necrosis
Tx: hydroxyurea, bone marrow transplant
-
Autoimmune Hemolytic Anemia: Describe
Extrinsic Hemolytic Normocytic Anemia
Many different disease: Erythroblastosis Fetalis, EBV, SLE, CLL, drugs...
Whenever the immune system decides to attack RBC's - autoimmune hemolytic anemia
-
Microangiopathic Anemia: Defect, Findings
RBCs damaged when going through small vessel.
DIC, TTP/HUS, SLE, Malignant HTN.
Schistocytes
-
Macroangiopathic Anemia: Defect and findings
Prosthetic Heart Valves
Big Vessels
Schistocytes
-
What are two protozoal diseases that can cause hemolytic anemia?
-
In what hematologic disorder would you find the following abnormal tests?
Ham's Test
DEB test
Heinz Bodies
Basophilic Stippling
Osmotic Fragility Test
- Hams - PNH
- DEB - Fanconi's Anemia
- Heinz - G6PD
- Basophilic stippling - Lead Poisoning, Iron Deficieincy, ACD, Thalassemia
- Osmotic Fragility Test - Hereditary Spherocytosis
-
Platelets: Derived from _, life span of _
-
What is the vWF receptor on a platelet? What is the fibrinogen receptor?
- vWF - GpIb
- Fib - GpIIb/IIIa
-
Name 4 Platelet Disorders
- Bernard Soulier Disease
- Glanzmann's Thrombasthenia
- Idiopathic Thrombocytopenic Purpura
- Thrombotic Thrombocytopenic Purpura
-
Bernard-Soulier Disease: PC, BT, Defect
ADHESION DEFECT
Platelet Count decreased, Bleeding Time increased
defect in Gp1b (can't bind vWF)
-
Glanzmann's thrombasthenia: BT, Defect
AGGREGATION defect
BT increased
Defect in GpIIb/IIIa
*No Thrombocytopenia
-
Idiopathic Thrombocytopenic Purpura: PC, BT, Defect
Decreased PC, Increased BT
Autoantibodies to GpIIb/IIIa - platelet destruction
See Increased Megakaryocytes
Tx: Steroids or IV IgG
-
Thrombotic Thrombocytopenic Purpura: PC, BT, Defect, Labs, Symptoms
AGGREGA TION
PC decreased, BT increased
Defect ADAMTS 13 - can't degrade vWF
Labs: schistocytes, increased LDH
- Symptoms: Pentad
- 1) Neuro
- 2) Renal
- 3) Fever
- 4) Thrombocytopenia
- 5) MIHA (microangiopathic hemolytic anemia)
Very closely related to Hemolytic Uremic Syndrome (HUS)
-
Hemolytic Uremic Syndrome: Symptoms and bug association
- 1) Kidney Involvement!
- 2) Neuro
- 3) Fever
- 4) Hemolytic Anemia
- 5) Thrombocytopenia
-
von Willebrand's Disease: MOA and Tx
ADHESION defect
MOA: Intrinsic Pathway coagulation defect, autosomal dominant
Tx: DDAVP releases vWF stored in epthelial cells
-
DIC: Tests, MOA, Causes, Labs
Tests: Decreased PC, increased BT, PT and PTT
MOA: widespread clotting activation -> depletes clotting factors -> bleed out
- Causes: STOP Making New Thrombin:
- S - Sepsis
- T - Trauma
- O - Obstetric Complications
- P - Pancreatitis
- M - Malignancy
- N - Nephrotic Syndrome
- T - Transfusion
Labs: Schistocyes, D dimers, decreased fibrinogen, factors V and VIII.
-
What pathway does PTT correspond to? PT?
- PT - Extrinsic. "The EX PresidenT went to WARfarin"
- PTT - Intrinsic
-
What is PTT? What is PT?
PTT - Partial Thromboplastin Time.
PT - Prothrombin Time
-
NSAIDS inhibit what substance important in platelet aggregation?
Thromboxane A2
-
Aspirin: MOA, Clin Use, Tox
MOA: irreversible COX inhibition
Clin Use: anti-platelet
Tox: Gastric Bleeding, Reye's Syndrome, Tinnitus
-
Clopidogrel and Ticlopidine: MOA, Clin Use, Tox
MOA: irreversible ADP receptor inhibition
Clin Use: ACS, coronary stenting,
Tox: neutropenia (ticlopidine)
-
Cilostazol, dypyridamole: MOA, Clin Use, Tox
MOA: Phosphodiesterase III inhibitor
Clin Use: claudication, stroke prevention, angina prophylaxis
Tox: flushing, abd pain
-
Abciximab: MOA, Clin Use, Tox
MOA: MAB binds GP IIb/IIIa - prevents aggregation
Clin Use: ACS
Tox: Thrombocytopenia
-
Tirobifan and Eptifibatide: MOA
binds GpIIb/IIIa
-
Leukemia vs. Lymphoma
Leukemia - bone marrow involvement. Tumor cells in blood
Lymphoma - tumor from lymph nodes
-
Leukemoid Reaction: define
increased WBC and leukocyte alk phos due to infection, not neoplasia
-
Hodgkin vs. Non Hodgkin Lymphoma
- Hodgkin:
- Reed-Sternberg Cells
- Local Single Group of Nodes
- Low Grade Fever, Night Sweats, Weight Loss
- Non-Tender Mediastinal Lymphadenopathy
- 50% EBV associated
- Bimodal Distribution Young or Old
- Non Hodgkin:
- HIV and Immunosuppression
- B Cell Lymphomas
- Middle Age
-
What is the most common lymphoma in US adults?
Diffuse Large B cell lymphoma
-
What lymphoma is associated with Reed Sternberg Cells?
Hodgkin Lymphoma
-
What two lymphomas are associated with EBV?
Burkitt and Hodgkin
-
What is the lymphoma equivalent of CLL?
small lymphocytic lymphoma
-
What disease has the "starry-sky" pattern due to phagocytosis of apoptotic tumor cells?
Burkitt Lymphoma
-
What hematological disease is associated with Sjogren syndrome, Hashimotos thyroiditis and H. pylori?
Marginal cell MALToma
-
What is the most common type of Hodgkin Lymphoma?
Describe RS ratio, prognosis.
Nodular Sclerosing, Low RS ratio
Excellent prognosis
-
Burkitt's Lymphoma: Age group, Genetics, Path
Non-Hodgkin Lymphoma
young adults
t (8:14), c-myc
Starry Sky Appearance, Associated with EBV, Jaw Lesion, co infection with HIV
-
Diffuse Large B Cell Lymphoma
Non Hodgkin Lymphoma
older adults
Most common adult NHL.
-
Mantle Cell Lymphoma: Age, Genetics, Prognosis
Non Hodgkin Lymphoma
Older men
t (11:14), Deactivation of cyclin D
Poor prognosis
-
Follicular Lymphoma: Age, Genetics
NHL
Adults
t(14;18), bcl-2 expression (inhibits apoptosis)
-
Adult T Cell Lymphoma: Age, Genetics, Presentation
NHL
Adults
Caused by Viral infection HTLV-1
cutaneous lesion presentation. Aggressive
-
Acute Lymphoblastic Leukemia: Age, Details
"Blasts" = Acute
- Children
- Bone Pain
- Good Prognosis
- PAS+
- Associated with Down's Syndrome (We ALL go DOWN together)
-
Acute Myeloblastic Leukemia: Age, Details
"Blasts" = Acute
- Older Adults 60yo+
- Auer Rods
- PAS -
- t(15;17) -> M3 variant responds to retinoic acid
-
AML Risk Factors:
- Radiation, Benzene, Alkylating Agents
- Aplastic Anemia
- Down, Fanconi or Bloom Syndrome
-

Identify cell and associated disease
Reed Sternberg (Owl Eye)
Hodgkin Lymphoma
-

Identify cell and associated disease
"smudge cells"
CLL
-

Identify cell and associated disease
hairy cell leukemia
"hair cells"
-

Identify cell and associated disease
Auer rods seen in myeloblasts
AML
-
Chronic Lymphocytic Leukemia: Age, Cell types and markers
- Age: over 50
- B cell markers
- Smudge cells
- Warm and cold agglutinins
-
Chronic Myelogenous Leukemia: Age, Genetics, Symptoms and Treatment
Age: 30-60y/o
May progress to AML in 80% of cases
Philadelphia Chromosome is ALWAYS present (Philadelphia CreaML Cheese)
Fatigue, splenomegaly, "blast crisis" -> AML,
Tx: Imatinib
-
Name the 4 chronic myeloproliferative disorders
Polycythemia vera - abnormal clone of hematopoeitic stem cells
Essential Thrombocytosis - megakaryocytes go out of control
Myelofibrosis - fibrotic obliteration of bone marrow (Teardrop cell)
CML - Philadelphia Chromosome
-
Langerhans Cell Histiocytosis: Path and Genetics
Proliferative disorder of dendritic cells
Birbeck Granules "tennis rackets" on EM
Cells express S100 (skin and neuro) and CD1a
-
t(9;22) associated with:
CML - Philadelphia Chromosome
'Tx: Imatinib
-
t(8;14) associated with:
Burkitt's Lymphoma (c-myc)
-
t(14;18) associated with:
Follicular Lymphomas (bcl-2 activation)
-
t(15;17) associated with:
M3 AML - auer rods, DIC, trans retinoic acid
-
t(11;22) associated with:
Ewing's Sarcoma
-
t(11;14) associated with:
Mantle Cell Lymphoma
-
t(8;21) associated with:
AML - Down Syndrome (extra chance to mutate due to trisomy 21)
-
Multiple Myeloma: Associations, Genetics, Age
Most common tumor of bone in elderly
- Lytic lesions
- Bence Jones proteins in urine (not on basic UA)
- Rouleaux Formation (Stacks of RBCs)
- "Clockface Chromatin"
- Monoclonal plasma cell "fried egg" appearance - shitloads of one type of antibody
CRAB Symptoms: Hypercalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back Pain
-
MGUS:
monoclonal plasma cell expansion without any symptoms
-
Most common leukemia in children
ALL
-
Most common leukemia in adults
CLL
-
AML associated with Down Syndrome
t8;21, AML M7
-
AML that are CD13 and CD33+
M0-M6
-
AML with Characteristic Auer Rods
M3
-
Myelodysplastic syndromes have a tendency to progress to:
AML - blast crisis
-
Myeloproliferative disorders may progress to
AML
-
Greater than 20% blasts suggest:
Acute Leukemias ALL and AML
-
Leukemia with more mature cells and less than 5% blasts
Chronic Leukemias
-
AML that is CD41 and CD61 +
AML M7
-
-
Leukemia that commonly presents with bone pain
ALL
-
Lekemia equivalent of Burkitt Lymphoma
ALL L3
-
Numerous basophils, splenomegaly and negative for leukocyte alkaline phosphatse
CML
-
Most common neonatal leukemia
AML M7
-
Always positive for philadelphia chromosome
CML
-
Only AML that is CD13 and CD 33-
AML M7
-
Acute leukemia positive for peroxidase
AML
-
Solid sheets of lymphoblasts in marrow
ALL
-
PAS (-) acute leukemia
AML
-
Always associated with BCR-ABL genes
CML
|
|