heme part 2 Flashcards
What are some reasons for prolonged PTT
Heparin
DIC
vWD
Hem A/B
What is the treatment for Heparin overdose
Protamine Sulfate
What measuring the intrinsic pathway
PTT - I II V VIII IX X XI XII
1, 2, 5, 8, 9, 10, 11, 12
What measuring the extrinsic pathway
PT - I, II, V, VII, X
1, 2, 5, 7, 10
What are some reasons for prolonged PT
warfarin therapy
vitamin K
DIC
what is the treatment for warfarin overdose
Vitamin K
Key terms for this diagnosis- Thrombocytopenia, microangiopathic hemolytic anemia, neurologic (headache, stroke) symptoms, fever
Thrombotic thrombocytopenic Purpura - TTP
ADAMTS-13 is positive in what ?
Thrombotic thrombocytopenic Purpura - TTP
What labs are positive with Thrombotic thrombocytopenic Purpura - TTP
Thrombocytopenia - low pLT's Normal PT, PTT Hemolytic anemia The problem is not clotting factors this is how you can tell the difference between DIC
Treatment for Thrombotic thrombocytopenic Purpura - TTP
- Plasma
- steroids to suppress the immune system
- Cyclophosphamides
Do not give pLT’S it will cause CLOTS!
Key terms for this diagnosis- Thrombocytopenia, microangiopathic hemolytic anemia and kidney failure, children
Hemolytic Uremic Syndrome - HUS
what is Hemolytic Uremic Syndrome - HUS usually caused by?
Enterohemorrhagic E Coli 0157: H7
Shigella
Salmonella
Labs for HUS
Hemolytic anemia
BUN/Creatine
Treatment for Hemolytic Uremic Syndrome - HUS
- Plasma - FFP
2, antibiotics can make worse
Pathological activation of coagulation system leading to widespread micro-thrombi which consumes clotting proteinss V, VIII fibrinogen and plts
leading to severe thrombocytopenia and diffuse bleeding
Disseminated Intravascular Coagulation- DIC
What are some causes of DIC
Infections- Gram neg endotonixs
Maligancy- AML, Lung or GI, Prostate
OB- pre-eclampsia, abruptio placentae
burns, trauma, liver disease
what are symtoms of DIC
widespread hemorrhage- at blood draw sites, mouth nose bleeding
clots -> renal failure and gangrene
Labs for DIC Fibriogen PTT PT/INR PLTS RBCs shape
Fibriogen- decreased it is being used up PTT- proglonged PT/INR - proglonged PLTS- severely low RBCs shape - schistocytes Fibinolysis- D-dimer +
aquired abnormal isolated thrombocytopenia of unknown cause
Idiopathic autoimmune thrombocytopenia purpura- ITP
Acute ITP is most common in whom?
Chronic ITP ?
Acute- boys with viral infection and self limiting
chronic- adults often recurrent
S/S of ITP
often asymptomatic
mucous bleeding- purpura or bruises
petechiae, bullae, nose bleeds, hemorrhage, bleeding gums
what labs are positive in ITP
only low PLT’S
smear- megakaryocytoes or large sized platelets
Treatment for ITP
Children- observe
Adults- steroids, IVIG, splenectomy
Hemophilia A is what factor deficiency
factor VIII 8
Hemophilia B is what factor deficiency
factor XI 9 - Christmas disease
Key terms for this diagnosis- x-linked recessive trait occurs in males, Intrinsic pathyway affected, hemathrosis
Hemophilia A - Factor VIII 8 deficiency
Hemophilia A - Factor VIII 8 deficiency
labs
Prolonged PTT
normal PT
normal platelet levels
Treatment for Hemophilia A - Factor VIII 8 deficiency
- Factor VIII 8 & vWF
2. Desmopressin - DDAVP
Key terms for this diagnosis- X-linked recessive trait in males, deep tissue bleeding, prolonged PTT that corrects with a mixing study
Hemophilia B- IX 9 Christmas factor
Treatment for Hemophilia B
Factor IX 9 infusion only
Desmopressin DDAVP is not helpful
Key terms for this diagnosis- autosomal dominant disorder with ineffective platelet adhesion
most common hereditary bleeding disorder
Von Willebrand disease
What is necessary for initial platelet adhesion and prevents factor VIII 8 degradation
Von Willebrand disease
Labs positive with Von Willebrand disease
VwF levels decreased
PTT prolonged
PTT will correct with a mixing study
Treatment for Von Willebrand disease
Type I: desmopressin, cryoprecipitate
Type II: DDAVP + Von willebrand factor and factor 8
Type III: Von willebrand factor and factor 8
Lymphoctye neoplasm that is associated with EBV, upper body lymph noes most common
Hodgkins Disease
Hodgkins lymphoma
What are Pel-Ebstein Fevers
what disease?
Cyclical fevres that increase and decrease over a period of 1-2 weeks
night sweats are present
key terms for this diagnosis- male with enlarged lymphnodes in upper body, fevers that come and go, night sweats, weight loss
Hodgkins lymphoma
Reed Sternberg cells are seen in what disease
Owl-eye appearance
Hodgkins lymphoma
What are some work-up tests to do for Hodgkins lymphoma
- CT scan or PET for staging
2. Blood cells- Reed sternberg cells
Treatment for Hodgkins lymphoma
- radiation and chemo
* Highly curable
What are the differences between Hodgkins and non-hodgkins lymphoma
Hodgkins- YOUNGER, upper lymph nodes
Non- OLDER Peripheral lymph nodes (GI, skin, CNS)
what is Burkitt lymphoma?
Type of Non-Hodgkin lymphoma
that starts with abdominal pain
Treatment for Non-Hodgkin Lymphomas
- Follicular- not curable
- Large B-cell type- aggressive but curable with
Chemo and radiation - Rituxamab
Proliferation of a single clone of plasma cells
increase monoclonal antibodies like IgG or IgA
Multiple Myeloma
Key terms for this diagnosis- AA man older, bone pain at spine or ribs, recurrent infections, elevated calcium, anemia and kidney failure
Multiple Myeloma - plasmacytoma
Multiple Myeloma - “BREAK”
what does it stand for based on the symptoms
B- bone pain
R- Recurrent Infections
E- Elevated calcium from bone destruction
A- Anemia
K- Kidney failure due to proteins in the kidney it can deposit
what is seen on labs with Multiple Myeloma
serum electrophoresis- monocolonal protein spike
Urine electrophoresis- Bence-jones proteins
blood smear - rouleaux formation
What will an x-ray show with MM
Punched-out leasions
Treatment for Multiple Myleoma
Stem cell transplant
with chemotherapy
what is the most common childhood malignancy
Acute Lymphocytic leukemia - ALL
Key terms for this diagnosis- 3-7 years with pancytopenia, fatigue, lethargy, bone pain, headaches, stiff neck, visual changes, , hepatosplenomegaly
lymph-node enlargement
Acute Lymphocytic leukemia - ALL
What will a blood smear show with Acute Lymphocytic leukemia - ALL
> 20% blasts
WBC 5-100K
anemia
PLT’S low
Treatment for Acute Lymphocytic leukemia - ALL
Oral chemotherapy
good prognosis 90% remission
What is the most common leukemia in adults
Chronic Lymphocytic Leukemia - CLL
Key terms for this diangosis- often asymptomatic but seen on blood tests, fatigue, increased infections, lymphadenopathy, hepatospleomegaly,
smear- well differeniated lymphocytes with smudge cells
Chronic Lymphocytic Leukemia - CLL
Treatment for Chronic Lymphocytic Leukemia - CLL
- observe
- chronic- chemotherapy
- if in blast crisis- Allopurinol
What is the most common ACUTE leukemia in adults
Acute Myeloid leukemia - AML
Key terms for this diagnosis- acute anemia, throbocytopenia, neutropenia, splenomegaly, gingival hyperplasia and bone pain
Leukostatsis >100,000, blood smear shows Auer rods with>20% blasts
Acute Myeloid leukemia - AML
Treatment for Acute Myeloid leukemia - AML
- Combination chemotherapy
* risks of tymor lysis syndrome can kill them due to a large number of cells being destroyed
Key terms for this diangosis- asympatomatic until blastic crisis, labs show WBC 100,000, Philadelphia chromosome +
Chronic Myeloid Leukemia - CML
Treatment for Chronic Myeloid Leukemia - CML
- Chemotherapy in Philadelphia +
Acquired Myeloproliferatvie disorder with overproduction of 3 stem cell lines, mostly RBC’s but increased WBC and PLT’s
Polycythemia Vera
Key terms for this diangosis- Older Man with Jak2 mutation, Increased HCT with no hypoxia, pruritius with a hot bath, facial flushing, episodic burning/throbbing of hands and feet with edema
Polycythemia Vera
Treatment for Polycythemia Vera
- Phlebotomy -> do until HCT is less than 45
2. Hydroxyurea - will stop cells that divide quickly
Key terms for this diangosis- obese smoker or a normal person with hypoxic environment with high HCT but normal WBC and plts
Secondary Erythrocytosis
Genetic disorder with excess iron deposits in the heart, liver, pancreas. Increased intestinal iron absorption
Hereditary Hemochromatosis
Key terms for this diangosis- 40years, iwht abd pain cirrhosis, fatigue, cardiomyopathy, arrhythmias, tesicule atropy/immpotence, metallic or bronze skin
Hereditary Hemochromatosis
iron overload will show the bronze skin
“bronze diabetes”
What is the gold standard test for Hereditary Hemochromatosis
Liver biopsy for increase hemosiderin
Treatment for Hereditary Hemochromatosis
- Phlebotomy
test relatives
genetic couseling
no iron pills alcohol or vitamin C
Key terms for this diagnosis- cirrhosis, end stage liver disease with bleeding
increased PT/PTT that corrects with mixing study, low albumin. decreased fibrinogen and PLTS
Coagulopathy of advanced liver disease
the liver is failing and cant not make coagulation factors
Treatment for Coagulopathy of advanced liver disease
- FFP
2. Cryo will contain fibrinogen, factor VIII 8 and vWF
Most common inherited cause of Hypercoagulability
Factor V leiden
Key terms for this diagnosis- young patient with a DVT or PE with no precipitating factor
Factor V leiden
Treatment for Factor V leiden
- high risk- Anti coagulation always
2. moderate risk- give anticoagulants during high risk procedures
Protein C deficiency will lead to what problem?
increased risk of recurrent DVT’s and PE’s
Protein C is needed for fibrinolysis and clot lysis
If a patient with Protein C deficiency is given heparin what is a common side effect they experience
Warfarin-induced skin necrosis
Antihrombin III deficiency will lead to what problem?
recurrent DVT’s and PE’s
first episode often occurs between 20-30years