Hematology exam review (summer) Flashcards
Hgb number in anemia?
Male <12
Female <15
Where is iron absorbed?
Duodenum
What conditions is iron absorbed?
Acidic conditions
Which iron study reflects total body stores of iron? Normal value?
Serum Fe
>30 is normal
Men and non-menstural women need how much iron/day?
1mg/day
Menstural women need how much iron/day?
3-4mg/d
Preggers women need how much iron/day?
2-5mg/d
Normal Recitulocyte Count range?
0.5-2%
What does Reticulocyte Count measure?
Bone marrow function.
High Reticulocyte Count means what type of cells being produced?
Immature cells
Normal Reticulocyte Index Count range?
2-3
Normal Serum B12? Deficiency?
Normal >210
Deficiency <170
Normal RBC Folic Acid level? Deficiency?
Normal >140
Deficiency <140
What is #1 MC anemia?
Iron Deficiency Anemia
MC cause of Iron Deficiency Anemia?
BLEEDING (menstural or GI)
How often screen 12-18 y/o F (non-preggers) for Iron Deficiency Anemia?
q5-10 year
When to first screen preggers for Iron Deficiency Anemia?
At first prenatal visit
When to screen men and post-menopausal F for Iron Deficiency Anemia?
No screening
What very weird craving in Iron Deficiency Anemia?
Pagophagia (ice craving)
What happens to tongue, nails, and corners of mouth in severe Iron Deficiency Anemia?
Brittle nails, nail spooning (koilonychia), and cheilosis of mouth corners
How much iron lost from menstural bleed?
1mg/day
How much iron lost from preggers, delivery, and nursing?
1g/d
How much iron lost per 100mL whole blood?
50mg Fe per 100mL whole blood
CBC and Iron Deficiency Anemia?
Hgb low, RBC low
TIBC up or down in Iron Deficiency Anemia?
Up. More binding places for iron due to not enough iron.
Serum Fe, Serum Iron, and % Sat in Iron Deficiency Anemia?
DOWN
Two essential lab tests for Iron Deficiency Anemia?
Hgb <12 or 15, Serum Fe <30
MCV and MCHC levels in Iron Deficiency Anemia?
Microcytic <80
Hypochromic <27
TOC for Iron Deficiency Anemia?
Ferrous Sulfate 325mg TID x1-2 mo until normal CBC
When to do CBCs in Iron Deficiency Anemia?
3 weeks (half better), 2 months (normal), 6 months (normal and stores rebuilt)
Tx of Iron Deficiency Anemia months 3-6?
325mg PO qd to rebuild stores
2 things which increase iron absorption?
- Empty stomach
2. Orange juice
SEs of Ferrous Sulfate?
Nausea, constipation, diarrhea, dark stool. Reduce dose.
When to use IV iron in Iron Deficiency Anemia?
Can’t absorb iron orally. IBD, gastric bypass, etc.
What is reduced in Thalassemia?
Reduced production of alpha or beta blobin chains
Alpha Thalassemia reduction of what?
Reduced alpha globin production
Who gets Alpha-1-Thalassemia? (two)
MC in S.E. Asia and China
Geneotype in Alpha-1-Thalassemia?
Heterozygous. Each parent missing one gene.
alpha ____/alpha ____
Who gets Alpha-2-Thalassemia?
MC black
Geneotype in Alpha-2-Thalassemia?
Homozygous. One parent missing each.
alpha alpha/____ ____
Genotype in Alpha Thalassemia Intermedia (aka Hgb H Dz)?
alpha ___/___ ___
Genotype of Alpha Thalassemia Majora (aka Hydrops Fetalis)?
No alpha globin at all
___ ___ / ___ ___
MCV in Alpha Thalassemia?
Microcytic
What does electrophoresis show in Alpha Thalassemia trait?
Normal Hgb.
What does electrophoresis show in Alpha Thalassemia Intermediate (Hgb H Dz)?
10-40% HgbH
S/Sx of Alpha Thalassemia trait?
No clinical S/Sx.
Tx of Alpha Thalassemia trait? Life expectency?
No tx. Normal expectency.
S/Sx of Alpha Thalassemia Intermediate (Hgb H Dz)?
Pallor, splenomegaly, fatigue, weakness
TIBC normal/increased/decreased in Alpha Thalassemia?
Normal or increased
Iron normal/increased/decreased in Alpha Thalassemia?
Normal or increased
Tx for Alpha Thalassemia Intermediate (Hgb H Dz)?
Folic Acid 1mg PO
What to avoid with Alpha Thalassemia Intermediate (Hgb H Dz)?
Avoid oxidative (sulfa) drugs, avoid iron
When to tranfuse PRBC in Alpha Thalassemia Intermediate (Hgb H Dz)? Goal Hgb?
If severely low Hgb. Goal 9-10.
Tx for refractory Alpha Thalassemia Intermediate (Hgb H Dz)? (Hint: take something out)
Splenectomy
When needed if getting regular transfusions for Alpha Thalassemia?
Iron chelation with Deferastrox 20-30mg/kg/d
Type of counseling needed for Alpha Thalassemia?
Genetic
Genetic bases of Beta Thalassemia?
Point delection on chromosome 11
Which globin chains are affected in Beta Thalassemia?
Beta-globin chains
What happens to Beta-globin chains in Beta Thalassemia?
Replaced with Beta-like chains (Delta and Gamma)
Beta Thalassemia MC in which population? (Hint: 2)
Mediterranian and Middle Eastern
Genotype of Beta Thalassemia Minor (aka Trait)? (Hint: two)
B/B+ or B/B˚
What percent are Beta-chains reduced in Beta Thalassemia Minor/Trait?
50%
S/Sx of Beta Thalassemia Minor/Trait?
ASx
CBC shows what in Beta Thalassemia Minor/Trait?
Clinically insignificant microcytic anemia
Electrophoresis shows what in Beta Thalassemia Minor/Trait?
HgbA2 4-8%
Tx required in Beta Thalassemia Minor/Trait?
No tx required
Counseling in Beta Thalassemia Minor/Trait?
Genetic counseling
Genotype of Beta Thalassemia Intermedia?
B+/B+
homozyous
When does Beta Thalassemia Intermedia begin to manifest?
6mo old when HgbF goes away
Where are bone deformities in Beta Thalassemia Intermedia?
Face
What type of fractures in Beta Thalassemia Intermedia?
Pathologic
Spleen and liver in Beta Thalassemia Intermedia?
Hepatosplenomegaly
When does severe anemia start in Beta Thalassemia Intermedia?
6 months
Three types of hemoglobin in Beta Thalassemia Intermedia?
HgbA 30%
HgbA2 10%
HgbF 6-100%
What does Peripheral Smear show in Beta Thalassemia Intermedia?
Bsophillic Stipling
Beta Thalassemia Majora aka?
Cooley Anemia
Growth and deformity in Beta Thalassemia Majora/Cooley Anemia?
Stunted growth, bone deformity
Spleen and liver in Beta Thalassemia Majora/Cooley Anemia?
Hepatosplenomegaly and jaundice
MCV in Beta Thalassemia Majora/Cooley Anemia?
Severe low
Majority of Hgb type in Beta Thalassemia Majora/Cooley Anemia?
HgbF
Tx of choice in Beta Thalassemia Majora/Cooley Anemia?
Allogeneic stem cell transplant
How often to transfuse PRBC in Beta Thalassemia Majora/Cooley Anemia?
Weekly
What to avoid in Beta Thalassemia Majora/Cooley Anemia?
Oxidative meds (sulfa, iron)
How to treat iron overload?
Deferoxamine
Prophyria due to what?
No porpherin ring in RBC
Skin in Prophyria?
Very pale skin
Light exposure does what in Prophyria?
Burns, blisters
Teeth in Prophyria?
Vampire-like teeth
Anemia of Chronic Dz due to what?
Conditions which reduce iron uptake
EPO response in Anemia of Chronic Dz?
Down
S/Sx in Anemia of Chronic Dz?
Fatigue, weak, dizzy
Hepcidin levels in Anemia of Chronic Inflammation?
Increased!
Serum Iron levels in Anemia of Chronic Inflammation?
Low
Serum Fe levels in Anemia of Chronic Inflammation?
Normal or inc
RBC life and mass in Anemia of Chronic Dz?
Decreased
Tx for Anemia of Chronic Dz?
Tx underlying condition
Folic Acid and/or Iron def
When to transfuse PRBC in Anemia of Chronic Dz?
If severe anemia
What med to give in Hgb <10 in Anemia of Chronic Dz? Goal Hgb?
Recombinant EPO until 10-12
Shape of Sideroblast in Sideroblastic Anemia?
Ringed sideroblast.
Where is ringed sideroblast found in Sideroblastic Anemia?
Bone Marrow
Iron levels in Sideroblastic Anemia?
Too much iron. Can’t get into RBC.
4 causes of acquired Sideroblastic Anemia?
- Cu deficiency
- INH
- Linezolid
- Chloramphenicol
Tx for INH acquired Sideroblastic Anemia?
B6 to fix
TIBC in Sideroblastic Anemia?
DOWN
SI, SFe, and % Sat in Sideroblastic Anemia?
Up!
Dx for Sideroblastic Anemia?
Bone marrow biopsy
Why splenectomy in congenital Sideroblastic Anemia?
Tertiary RBC production center.
Lead Toxcity does what to iron binding?
Decreases iron binding in proporphyrin ring
Chromic in Lead Toxcity?
Hypochromic
What type of cells seen on peripheral smear in Lead Toxcity?
Basophillic Stipiling
Basophillic Stipiling seen in what type of toxicity?
Lead Toxcity
Tx for Lead Toxcity?
Chelate
MCV in Alcoholic Anemia?
Macrocytic 100-110
How much EtOH per day to cause Alcoholic Anemia?
80g/day (1 bottle of wine)
Tx for Alcoholic Anemia? When resolves?
Stop drinking. Resolves in 2-4 months.
Myelodysplastic Syndromes are due to an inappropriately low response to what?
Reticulocytes
Which population has 2-3x higher instance of Aplastic Anemia?
East Asian
Ages of Aplastic Anemia?
Biphasic. 10-25, >60.
Aplastic Anemia due to injury of what?
Pluripotent stem cells in BM. Impairs proliferation and differentiation.
What is low in Aplastic Anemia?
Low PLT, RBCs, WBCs
Causes of Aplastic Anemia?
Viruses, drugs, chemo, radiation, toxins
What does CBC show in Aplastic Anemia?
Pancytoanemia
What does Peripheral Smear show in Aplastic Anemia?
Look normal but reduced number
Reticulocyte Count/Index increased or decreased in Aplastic Anemia?
Decreased
Tx for Aplastic Anemia under 20 y/o?
Allogenic hepatopoietic stemm cell transplant
Tx for Aplastic Anemia under 50 y/o?
Immunisuppressive therapy
Most common type of Aplastic Anemia?
Fanconi Anemia
Fingers and Fanconi Anemia?
Sindactyly (fused digits)
Where is B12 absorbed?
Terminal Ileum
What is B12 a cofactor for synthesis of? (Hint: 2)
- Nucleic acid synthesis
2. Myelin synthesis
How long are B12 stores good for?
3 years
Causes of B12 anemia?
Vegans, Pernicious Anemia, Chron’s DZ, EtOH
Pernicious Anemia what type immune disease?
Autoimmune.
What is destroyed in Pernicious Anemia by Abs?
Parietal cells and intrinsic factor
MCV in Pernicious Anemia?
>
- Megaloblastic anemia.
Blood smear in Pernicious Anemia?
Hypersegmented neurophils and macro-ovalcytes.
Sx in Pernicious Anemia?
Anemia, pale, neuro symptoms (vibration, proprioception, memory)
When do Sx develop in Pernicious Anemia?
About 3 years
Normal Serum B12? When deficient?
Normal >210
Deficient <170
Tx for Pernicious Anemia?
VitB12 100mcg SC/IM qd x1wk, then 1/wk, then monthly for life
When do check CBC and B12 levels in Pernicious Anemia?
1-2 wk, 2 mo, then periodic indefinitely
What is the natural form of Folic Acid called?
Folate
Daily intake of Folic Acid is preggers or lactating?
600-800mcg
Folic Acid absorbed where?
Duodenum and jejunum
Folic Acid in what foods?
Fruits and veggies
How long are stores of Folic Acid?
2-3 months
MC cause of Folic Acid decifiency?
Inadequate intake. (Then EtOH abuse.)
MCV in Folic Acid decifiency?
Megaloblastic. MCV >115.
When do Sx appear in Folic Acid decifiency?
4-5 months
Tx for Folic Acid decifiency?
Folic Acid 1mg PO qd x1-4mo or until hematologic recovery
Expected response from tx in Folic Acid decifiency?
Rapid improvement and sense of well-being
When will see reticulocytosis after tx in Folic Acid decifiency?
5-7d
Sickle Cell is autosomal recessive or dominant?
Recessive
Hgb type in Sickle Cell? Which Hgb completely absent?
HgbS
HgbA absent.
AA substitution in Sickle Cell? In which globin chain?
Glutamic Acid becomes Valine in Beta-globin chain.
When do HgbS change into and do poorly when deoxygenated?
Poorly soluable. Turn into sickle shape.
HgbS RBC lifespan?
10-20 days.
Normal HgbA is 100 days.
What does Sickle Cell cause in microcirculation?
Recurrent vasoocclusion
What is a Sickle Cell Crisis? What causes it?
Acute episode with many sickle cells. Due to Infection, hypoxia, dehydration, acidosis, exercise at high altitude
What bacteria type due to bad spleen in Sickle Cell?
Encapsulated (pneomococcal, h flu, meningitis)
What is Chest Syndrome in Sickle Cell?
Lung injury and infarction due to occlusion
DX for Sickle Cell?
Hgb electrophoresis. Shows HgbS.
Daily tx for Sickle Cell?
Folic Acid 1mg PO qd
What to avoid in Sickle Cell?
Precipitation factors
What to give in acute crisis in Sickle Cell?
IV hydration, oxygen, and lots of analgesia for pain.
Tx for intractable pain or severe chest syndrome in Sickle Cell?
Exchange transfusion
What does Hydroxyurea do for Sickle Cell?
Increased production of HgbF
Tx for children with Sickle Cell?
Allogenic hematopoietic stem cell transplant
G6PD deficiency MC in?
Back males
What do G6PD enzymes do?
Maintain RBC membrane from oxidative stress
Does G6PD deficiency cause immune or non-immune hemolytic anemia?
Non-immune
G6PD deficiency due to what?
X-linked mutation. RBC damage triggered by Oxidative drugs, infection, or fava beans.
How often is hemolytic anemia in G6PD deficiency? (Constant, episodic, rarely, never?)
Episodic
What drugs to avoid in G6PD deficiency?
Oxidative drugs.
Bactrim, Dapsone (TB and Leprosy, antimalarials, INH, etc.
Coombs test in G6PD deficiency?
Negative
When will G6PD enzyme assay be low in G6PD deficiency?
During or after hemolysis
Peripheral Smear shows what type of cells in G6PD deficiency?
Shistocytes with bite or blister fragments
Tx for G6PD deficiency?
Self-limiting. Avoid oxidating drugs and fava beans. Transfuse PRBC when severe or symptomatic anemia.
Hemolytic Anemia do to what?
RBC destruction
Do Petechia and Purpura blance?
No
What does increased indirect bilirubin cause?
Jaundice (yellowed eyes and skin)
Dark urine due to what?
Increased bilirubin
Direct Coombs tests for what?
RBCs coated with Ab or complement
Indirect Coombs tests for what?
Ab against in RBCs in serum
MC Hemolytic Anemia?
Hereditary Sperocytosis
Hereditary Sperocytosis due to defect where in RBCs?
Membrane defect
Hereditary Sperocytosis and coombs test?
Negative! Not an immune issue!
Peripheral smart in Hereditary Sperocytosis?
Spherocytes
Tx for Hereditary Sperocytosis?
Supportive care and Folic Acid. Transfuse PRBC as needed. Splenectomy if severe.
Autoimmune Hemolytic Anemia cause?
IgG Ab bind to RBC membrane at room temp and activate complement to attack RBC membrane. “Warm”.
Which Coombs test in Autoimmune Hemolytic Anemia?
Positive direct coombs!
Tx for Autoimmune Hemolytic Anemia?
Steroids to blunt immune system. Splenectomy to remove site of RBC destruction. Plasma pheresis and transfusion.
What is Leukemia a malignancy of?
WBCs
Leukemia causes bone marrow to produce what number of WBCs?
Increased # of RBCs
What happens to cell lines in Leukemia?
Pancytopenia (Neutropenia, anemia, thromboytopenia)
Meyloid line gives rise to which WBCs?
Neutrophila
Eosinophils
Basophils
Which are the “granulocytes”?
Neutrophila
Eosinophils
Basophils
Lymphoid line gives rise to which WBCs?
B and T Lymphocytes
Which leukemia developes fast and has signs/symptoms- Acute or Chronic?
Acute leukemia
Speed of chronic leukemias? Sx?
Slow/insidious. ASx until progession or blast crisis.
Chronic Lymphocytic Leukemia (CLL) MC in who? (color and age)
White males, >65
Chronic Lymphocytic Leukemia malignancy of which WBC?
B Lymphocyte cells
Chronic Lymphocytic Leukemia S/Sx? Speed?
Slow onset. Often ASx.
Dx for Chronic Lymphocytic Leukemia?
> 5000 absolute B-Lymphocutes in peripheral smear >3mo
Tx for Chronic Lymphocytic Leukemia?
Only tx if +sx.
Cure for Chronic Lymphocytic Leukemia?
Allogeneic stem cell t/p
What is the MC childhood malignancy?
Acute Lymphocytic Leukemia “ALL”
Acute Lymphocytic Leukemia malignancy of which WBCs?
Clonal proliferation of immature B and T Lymphocytes in BM.
Onset of Acute Lymphocytic Leukemia?
Rapid onset
S/Sx in Acute Lymphocytic Leukemia?
Fever, fatigue, pallor
Anemic picture in Acute Lymphocytic Leukemia?
Pancytopenia/Anemia
DX for Acute Lymphocytic Leukemia?
BM Biopsy showing hypercellular >20% blasts
When to transfuse PRBC in Acute Lymphocytic Leukemia?
Hgb <8
Tx for Acute Lymphocytic Leukemia?
Chemo.
When to do BM transplant in ALL?
If relapses
Philadelphia Chromosome in which leukemia?
Chronic Myeloid Leukemia
Chronic Myeloid Leukemia malignant proliferatin of which WBC line?
Granulocytic/Meyloid line (N.E.B)
Chronic Myeloid Leukemia MC in what age?
> 50 y/o
Speed of onset in Chronic Myeloid Leukemia?
Slow
Chronic Myeloid Leukemia asx until what?
Blastic crisis
WBC number in Chronic Myeloid Leukemia?
> 100,000
Age of granulocytes in Chronic Myeloid Leukemia?
Increased number of old granulocytes with low or no leukocyte alk phos
Tx for Chronic Myeloid Leukemia?
Treat anemias. TKIs.
WHat is MC leukemia in adults?
Acute Myeloid Leukemia
What percent of blasts in BM with Acute Myeloid Leukemia?
> 20% blasts in BM
Time of onset in Acute Myeloid Leukemia?
Rapid onset
Bleeding in Acute Myeloid Leukemia?
Mucocutaneous bleeding. Petechiae and ecchymosis.
Acute Myeloid Leukemia and gingiva
Gingivitis
Peripheral smear shows what kind of cells in Acute Myeloid Leukemia?
Auer Rods
Auer Rods in which leukemia?
Acute Myeloid Leukemia
Tx for Acute Myeloid Leukemia?
Chemo + bone marrow transplant
Lymphoma is a malignancy of which tissues?
Lymphatic tissue
2 MC lymphatic tissues?
Lymphnodes, spleen
2 types of Lymphoma?
- Hodgkins Lymphoma
2. Non-Hodgkins Lymphoma
Reed-Sternberg Cells seen in which Lymphoma?
Hodgins Lymphoma
MC age for Non-Hodgkins Lymphoma?
> 50 y/o
2 types of Non-Hodgkins Lymphoma?
- Indolent
2. Aggressive
S/Sx of Indolent Non-Hodgkins Lymphoma?
Slow, waxing/waning s/sx. Hepatosplenomegaly. Painless lymphadenopathy. B Symptoms.
Is the lymphadenopathy in Lymptoms painful or painless?
PAINLESS
What are the B-symptoms in Lymphoma?
Fever, night sweats, weight loss
S/Sx of Aggressive Non-Hodgkins Lymphoma?
Acute, rapidly growing painless lymphedenopathy. Hepatosplenomegaly. Extranodal involvement on GI, skin, CNS
Dx of Non-Hodgkins Lymphoma?
Lymph node and tissue biopsy
What test to stage Non-Hodgkins Lymphoma?
PET/CT
Stage 1 Lymphoma how many lymph nodes?
Single lymph node
Stage 2 Lymphoma how many lymph nodes?
≥2 lymph nodes on same side of diaphragm
Stage 3 Lymphoma how many lymph nodes?
Both sides of diaphragm
Stage 4 Lymphoma how many lymph nodes?
In extra-lymphatic sites (liver, lungs, B.M.)
Hodgkins Lymphoma MC in people with what other dz?
HIV
Ages who get Hodgkins Lymphoma?
25-30, >55 y/o
Type of cells shown on excisional biopsy of Hodgkins Lymphoma?
Reed-Sternberg Cells
Multiple Myeloma is malignant proliferation of which cells?
Plasma cells d/t overproduction of monoclonal immunoglobulin
Multiple Myeloma d/t over production of what?
Monoclonal immunoglobulin (esp IgA and IgG) -> increased plasma cells
Where do plasma cells accumulate in Hodgkins Lymphoma?
Bone Marrow
MC bone malignancy?
Multiple Myeloma
Multiple Myeloma age of onset?
4-6th decade of life
Exposure to what two big things increase risk of Multiple Myeloma?
Radiation and pesticide exposure
Where is pain in Multiple Myeloma?
Bone pain. MC in spine and ribs.
What is bone pain due to in Multiple Myeloma?
Boney lytic lisons
What “formation” seen on CBC in Multiple Myeloma?
Roulex Formation (RBCs sticking together)
Calcium and Multiple Myeloma?
Increased serum Ca2+ d/t lytic lesions
ESR in Multiple Myeloma?
Increased
Tx for Hypercalcemia and pathologic fx in Multiple Myeloma?
Bisphosphonates
Tx for Multiple Myeloma?
Hematopoietic stem cell transplant + chemo
Hemophilia A is a deficiency of which factor?
Factor 8
Which pathway does Hemophilia A affect?
Instrinsic pathway
Lack of Factor 8 causes what to not form?
Thrombin
MC hemophilia?
Hemophilia A
Where does bleeding MC occur in Hemophilia A?
Weight-bearing joints. “Hemarthosis”.
Hemophilia A and aPTT time?
Prolonged!
How to dx Hemophilia A?
Very low F8 levels. <10IU.
How to treat mild Hemophilia A?
Demopressin. Releases stored Factor 8 from endothelium
Emergent tx for Hemophilia A?
IV Fresh frozen Plasma
Office treatment for severe Hemophilia A?
Recombinant Factor 8
Who gets Hemophilia A?
Males only. X-linked.
Hemophilia B due to lack of which factor?
Factor 9 deficiency
Where is bleeding in Hemophilia B?
Deep tissue bleeding
Hemophilia A and B bleed more or longer?
More, not longer.
Hemophilia B and aPTT?
Prolonged PTT
Office tx of Hemophilia B?
Factor 9 infusion
Emergent tx of Hemophilia B?
IV FFP
Hemophilia C due to which Factor?
Factor 11 decifiency
Disseminated Intravascular Coagulation due to sort of activation?
Pathological activation of coagulation system
What does pathological activation of coagulation system lead to in DIC?
Widespread microthrombi which use up coagulation proteins
Widespread microthrombi which use up coagulation proteins leads to what in DIC?
Severe thrombocytopenia and bleeding
What caues the pathological activation in DIC?
Infx, malignancy, OB issues, trauma/burns, rattlesnake bites
When to transfuse platlets in DIC?
If <20k
How to replace coag factors in DIC?
IV FFP
How to replace Fibrinogen in DIC?
Cryopercipitate
What to use for thrombosis in DIC?
Heparin
Polycythemia Vera causes what to be overproduced?
RBCs
Viscosity in Polycythemia Vera?
Increased
Polycythemia Vera age of onset?
60 y/o
What is Erythromelalgia in Polycythemia Vera?
Burning pain in hands and feet d/t increased PLT number and aggregation. Red/blue skin color.
Aquagenic Pruritus and Polycythemia Vera?
Itching after warm water exposure
Hgb, HCT, WBC, and PLT levels in Polycythemia Vera?
Increased
EPO levels in Polycythemia Vera?
Decreased!
Goal of tx in Polycythemia Vera?
Decreased thrombotic complications. Give 81mg ASA.
Phlebotomy target in Polycythemia Vera?
<45%
Idiopathic Thrombocytopenia immune or non-immune?
Autoimmune. Ab destroy megakaryocutes.
Idiopathic Thrombocytopenia MC in?
Kids s/p viral infection
Where is bleeding in Idiopathic Thrombocytopenia
Gingival and mucosal bleed
Rash in Idiopathic Thrombocytopenia?
Purpuric rash with petechiae.
Tx in Idiopathic Thrombocytopenia?
High dose glucocorticoids to blunt immune response.
Tx of Idiopathic Thrombocytopenia if severe?
IVIG
Tx of refractory Idiopathic Thrombocytopenia?
Splenomegaly
Thombotic Thombocytopenic Purpura due to what being destroyed?
Enzyme ADAMTS12
What does enzyme ADAMTS13 do?
Cuts large vonWillibrant Factor
What happens to RBCs in Thombotic Thombocytopenic Purpura?
Damaged due to microthombi. Causes intravasular hemolysis and shistocyte formation.
Pentad of S/Sx in Thombotic Thombocytopenic Purpura?
- Bruising and purpura
- Jaundice
- Neuro problems (HA, AMS, CVA, hallucinations)
- Renal failure
- Fever
PLT count in Thombotic Thombocytopenic Purpura?
Very low PLT count
TOC in Thombotic Thombocytopenic Purpura?
Early plasmapheresis qd
2 major roles of von Willenbrand Factor?
- Mediates adhesion of PLTs to site of vascular injuey
2. Binds and stabilize Factor 8
What is MC herediatary coagulation abnormality?
von Willenbrand Disease
von Willenbrand Disease d/t what?
Deficiency of vWF
MC type of von Willenbrand Disease?
Type 1= decreased vWF levels
S/Sx of Type 1 von Willenbrand Disease?
Asx or mild
Type 2 vW Disease due to what?
Quality defect of vWF
Type 3 vW Disease due to?
Complete absence of vWF -> decreased Factor 8 levels
Where bleed and bleeding time in von Willenbrand Disease?
Mucosal bleed- epistaxis, gingival, and menorrhagia.
Increased bleeding time.
PTT normal or prolonged in vW Disease?
Prolonged
Tx for moderate vW Disease?
Desmopressin
Tx for severe/Type 3 vW Disease
Virus inactivated vW Factor or cryopercipitate/FFP
PPX for surgery in vW Disease?
vWF-containine F8 concentrates
Heparin Induced Thrombocytopenia due to?
UFH exposure. Massive PLT decrease.
Type 1 HIT within how many hours of UFH explosure?
48h
Type 1 HIT immune or non-immune?
Non-immune
What will happen to PLTs in Type 1 HIT with continued exposure?
Normalize with continued UFH exposure
What happens to PLTs in Type 2 HIT?
PLTs activated after UFH exposure. CVA/MI/DVT/PE.
HIT risk decreased with which other heparin?
LMWH
Dx of Heparin Induced Thrombocytopenia?
HIT antibodies
Tx for HIT?
D/C UFH immediately
What to use instead of UFH for Warfarin bridge if have HIT?
Direct Thrombin Inhibitor (Dargatroban, Lepiridu)
When safe to switch to Warfarin in HIT if using Direct Thrombin Inhibitor?
When PLT >150,000
Tranfuse PLT in HIT?
NO! Risk Thrombosis!
HIT more than 100 days ago. OK to use UFH?
Yes
Warfarin’s MOA?
Decreases Vitamin K dependent clotting factors and decreases Vitamin K reserves
Warfarin indicated for?
DVT, PE, CVA, Afib, valve replacement
Warfarin dose?
2-10mg PO qd
Goal INR with Warfarin?
2-3
Warfarin’s inhibitors?
Vitamin K PO or IV.
Prothrombin Complex Concentrate > FFP
Warfarin CI’d with which 2 classes of anticoagulants?
- IIa inhibitors
2. Xa inhibitors
Warfarin PT education
- Inform all medical and dental providers
- Soft toothbrush
- No heavy contact sports
- No huge increase in leafy greens (contain VitK, can decrease INR)
- No new multivitamins, OTCs, or herbal supplements
- No preggers