Hematology Flashcards
___________ is the main component of RBCs that carry O2 and CO2
Hemoglobin
T/F: Hemoglobin levels may be lower in African American children
True
____________ is the ratio of RBC volume to whole blood volume
Hematocrit
______ _______ _______ is the measure of RBC size
Mean Corpuscular volume (MCV)
T/F: RBC’s are larger in adulthood than at birth?
False
Large at birth –> can remain larger through puberty
________ _________ ______ is the amount of hemoglobin in each RBC
Mean Corpuscular Hgb (MCH)
________ _______ _____ ________ is similar to MCH but measures the concentration of Hgb in each RBC and measures chromicity
Mean Corpuscular Hgb Concentration (MCHC)
What types of disorders/diseases can lead to microcytic hypochromic anemia?
THREE
Iron deficiency
Lead poisoning
Thalessemia
Anemias due to chronic inflammation result from failure to mobilize ______ from storage sites through the transferrin system, thus erythroid precursors are insensitive to _____.
Iron
EPO
__________ anemia is a very rare condition which is confirmed by the presence of ringed sideroblasts in the bone marrow
Sideroblastic Anemia
_________ hemolytic anemias result from a high reticulocyte response
Extrinsic (Aquired)
_________ hemolysis results from microangiopathic damage to RBCs.
What types of diseases are associaed with this?
Fragmentation Hemolysis
Can result from……
TTP
HUS
Giant Hemangioma
Artifical heart valves
_______-_______ hemolytic anemia is a hemolytic disease of a new born and is mediated by antibodies
Immune-mediated
What types of diseases or disorders are associated with immune-mediated hemolytic anemias?
Collagen Vascular Disease
Lymphoma
Drugs
What disorders or diseases are associated with normocytice anemias with a low reticulocyte response?
Inadequate bone marrow response / RBC aplasia
Transient Erythroblastopenia of Childhood (TEC)
Diamond Blackfan Anemia
Aplastic crisis
Aplastic crisis is typically transient and results from what virus?
Parvovirus B19
T/F: Aplastic crisis WOULD NOT result in significant exacerbation of anemia in patients with hemolytic diseases
False
It would
What history “factors / questions / information” is important to obtain when evaluating a patient for a possible anemia?
- Birth History (Prematurity)
- Family Hx
- Gender
- Diet
- Socioeconomic
- Travel Hx
- Drug Use
- Hx of Jaundice / Stones
- Systemic / Chronic Illnesses
Clinical presentation and Sx vary in patients with anemia but are typically dependent on the rate and reduction of ____.
Typically, physiologically disturbances are not seen until _______ is lower than ____
Hgb
Hgb
<7-8
What Sx may you see in a patient who is anemic?
Fatigue SOB Syncope Pallor Hypotension CHF Sx AMS Pica Tachycardia Anorexia Palpitations Vertigo Sore Tongue
What important lab values within a CBC should be checked when evaluating a patient for anemia?
Is one set of abnormal values enough to determine a patient’s anemia status?
Hgb
Hct
MCV
RBC#
No, these need to be abnormal on TWO draws
Other than a CBC what additional lab tests are helpful when evaluating a patient for anemia?
Reticulocyte count Peripheral Smear UA Comb's Test Guaiac stool
(Always remember the ‘pediatric rule of thumb’ in peds patients)
If you a NEWBORN is anemic what may likely be the cause of their anemia?
In early infancy?
6 mo - 12 yo?
Adolescence and beyond?
Newborn: Blood loss or hemolysis
Early Infancy: RBC Aplasia, Physiological anemia
6mo - 12yo: Nutritional anemia, inflammation, bone marrow infiltration
Adolescence: Iron Deficiency
_____ ________ is used for children with sickle cell disease who present with a fever of 101 or higher
Fever Protocol
T/F: Individuals with sickle cell disease have an increased risk of septicemia, especially in childhood
True
_______ is typically the herald of on of the acute complications of SCD such as ACS or Osteomylitis
Fever
What is a common source of infection in patients with SCD?
Venous Catheterization
In a SCD patient presenting with a fever, other than a history and physical, what should be immediately obtained?
CBC with Diff
Blood and urine Cx
A SCD patient with a fever of 103.1 or higher should be _________ for observation and given IV ___
Hospitalized
IV Abx
A febrile SCD patient presents with SOB, Dyspnea, Cough, and Rales…..
Other than CBC and Cx, what should be obtained?
CXR
Evaluating for acute ACS
A febrile SCD patient presents with multifocal bone tenderness accompanied with erythema and swelling…..
What is the most concernning DDx?
Acute bacterial osteomylitis
Immediately after blood Cxs are drawn on a febrile SCD patient what should be given?
What could be added in combination with this?
50 mg/kg of Ceftriaxone IV
15 mg/kg of Vancomycin can be added in combination in some patients
(Observe following this)
What is the admission criteria for febrile SCD patients?
What additional findings would raise concern and likely lead to admission?
- < 9-12 months old
- Problematic follow up
- Unstable during observation following ABx
Others….
Hx of sepsis Temperature >104 Pain Anemia Leukocytosis Luekopenia Thrombocytopenia
If a febrile SCD patient is able to be discharge from the ED, what needs to occur?
What should the patient’s parents be educated on?
Follow up within 24 hr with pediatrician of sickle cell doctor for re-check and 2nd ABx dose.
Patient’s parents need to be educated that if the patient worsens they need to return to the ED
Acute worsening anemia in patients with a history of anemia is classified by Hgb values less than what?
a Hgb >2 below known baseline
a Hgb <6 if the baseline is unknown
What are common causes of acute anemia?
- Associated w/ a vaso-occlusive crisis or ACS
- Associated w/ infection
- Hydroxyurea toxicity
- Delayed hemolytic transfusion reaction
- Aplastic episode (parvovirus B19, more common in children, adolescents)
- Splenic sequestration (more common in children, adolescents)
- Hepatic sequestration
- Acute blood loss due to papillary necrosis
If a patient is acutely anemic and presenting with symptoms of tachycardia, dyspnea, lightheadedness, hypoxia, and pain what should be given?
Simple Blood Transfusion
RBCs that are transfused should include matching for ___, __, and __ antigens
C, E, and K antigens
T/F: Transfusion should be avoided in a Hgb >10
True
Other than a Hgb >10, when should blood transfusion be avoided?
- Iron Overload
2. RBC Alloimmunization (Delayed hemolytic transfusion reaction, patients become non-transfusable)
This type of pain in SCD patients results in tissue damage often described as crushing, pounding, or squeezing
Inflammatory or nociceptive pain
This type of pain in SCD patients is a primary dysfunction of the nervous system that affects the somatosensory system resulting in damage to the peripheral or central nervous sytem
Neuropathic pain
Name the clinical charateristic of neuropathic pain in a SCD patient from the description below…..
- Exaggerated pain from a stimulus that normally produces pain
- Pain precipitated by a stimulus that is not normally painful
- Hypersensitivity
2. Allodynia
How is Neuropathic pain in a SCD patient typically described?
Burning Aching Cold Numb Radiating
_________ _________ pain crisis typically onsets suddenly (can be gradual) is triggered by cold, stress, or infection and manifests as severe pain in the chest, extremities, and in the back.
Vaso-occlusive
T/F: Labs, biomarkers, and imaging are able to include/exclude a pain crisis and validate the severity
False
Those can not
Are vaso-occlusive crisis patients at a high-risk for developing complications?
Yes
How is a vaso-occlusive crisis typically managed?
Analgesics
Typically opioids
The _____ _____ ____ is a scoring system used by more than half of the ED’s in the USA to triage SCD patients.
Emergency Severity Index
Level 2 = very high priority
A patient presenting with a vaso-occlusive crisis should receive analgesics within ____ minutes of triage or ___ minutes of registration
30
60
______ are commonly used to treat vaso-occlusive crisises if the patient has mild-moderate pain
NSAIDs
Should use transfuse just to treat pain in a SCD patient?
No
T/F: Incentive Spirometery should be encouraged during a pain crisis and ambulation should occur to avoid ACS
True
If a patient severely deteriorates during a vaso-occlusive pain crisis, what should you evaluate for?
Multi-organ failure
_______ _______ _______ is the exposure of blood to pro-coagulation such as tissue factor and cancer which forms fibrin in circulation leading to fibrinolysis and the depletion of clotting factors.
This ultimately leads to end organ damage.
Disseminated intravascular coagulation (DIC)
T/F: 25% thrombotic events in pts w/ inherited thrombophilia are associated w/ additional presence of an acquired risk factor
False
50%
Pts w/ >1 form of ______ thrombophilia or >1 from of _______ thrombophilia appear to have greater risk for thrombosis
Inherited
Acquired
What THREE anatomical disorders result in an increased risk for the patient to be in a hypercoaguable state?
- Paget-Schroetter syndrome (Subclavian/axillary vein compression)
- May-Thurner syndrome (Iliac vein compression syndrome)
- Congenital venous malformations of the inferior vena cava (Absent IVC syndrome)
What do patients in hypercoaguable states commonly present with?
DVT or PE
Remember Virchow’s Triad
Patients with DVT/PE should be treated with what type of anticoagulants and for how long?
Direct factor Xa inhibitors:
Rivaroxaban, Apixaban, Edoxaban
Indirect factor Xa inhibitors:
Enoxaparin, Fondaparinux
3 months (Occasionally 6 months in PE tx)
Acquired thrombophilia can occur as a result of what?
- Surgery / Hospitalization
- Malignancy
- Travel / Prolonged bed rest / Immobility
- Pregnancy
- OCPs
If a patient presents with any combination of the following….
Petechiae Ecchymosis Menorrhagia Epistaxis Bleeding gums Hemarthosis
What type of disorder should you suspect?
Bleeding disorder
What type of medications may result in a patients increased risk for bleeding?
- Anticoagulants
- NSAIDs
- Beta-lactam ABx
What labs would be most helpful in evaluating a patients bleeding risk?
- PT, aPTT
2. Platelet Count / Function test
What can be given to treat bleeding as a result of Warfarin?
Severe bleeding?
Bleeding as a result of Heparin?
Factor Deficiency?
vWB?
Warfarin: Vitamin K
Severe Bleeding: FFP
Heparin: Protamine
Factor Deficiency: Replace the factor
vWB: Intranasal ddAV
What is the most common inherited bleeding disorder?
von Willebrand Disease
Factor ___ deficiency would have a prolonged PT and normal aPTT
VII
Warfarin use or vitamin K deficiency would have a prolong ___ and normal ___
PT
aPTT
What factor deficiencys may be seen in a patient with a normal PT and a prolonged aPTT?
Factor VIII Deficiency (Hemophilia A)
Factor IX Deficiency (Christmas Disease)
Factor XI Deficiency
Factor XII Deficiency (will NOT cause bleeding)
If a patient has a normal PT and a prolonged aPTT what anticoagulant may have been administered?
Heparin
Disseminated intravascular coagulation (DIC) would have a ________ PT and aPTT
Prolonged
______ ______ is a macrocytic anemia that commonly results from EtOH abuse, poor diet, or malaborsption.
CBC: Megaloblastic anemia
MCV: High
Folate: <3 (Low)
Folate Deficiency
How is folate deficiency treated?
Supplemental oral folate
_____ __ ______ is a macrocytic anemia that commonly results from poor dietary history or gastric bypass with neuropathy.
CBC: Megaloblastic anemia, hypersegmented neutrophils
MMA: High
MCV: High
Folate: Normal
Vitamin B12 Deficiency
How is Vitamin B12 deficiency treated?
B12 Replacement (Typically IM)
Symptomatically, how can folate deficiency and vitamin B12 deficiency be distinguished from one another>
The presence of neurological symptoms would indicate Vitamin B12 deficiency
These include….
Loss of proprioception Vibratory Sense Swollen red tongue Anorexia Paresthsias
______ ______ occurs with vitamin B12 deficiency secondary to decreased intrinsic factor
Pernicious Anemia
What is a useful lab value in distinguishing between folate and vitamin b12 deficiency?
MMA (Methylmalonic acid)
This will be elevated in Vitamin B12 deficiency
Pernicious will have a positive _______ test with ________ levels of intrinsic factor
It will also have a ________ antibody to intrinsice factor
Schilling Test
Decreased intrinsic factor levels
Positive antibody
______ ______ is the most common cause of anemia
Iron Deficiency
What is the most common cause of iron deficiency anemia in Men?
Women?
Men: Chronic occult bleeding
Women: Menstrual blood loss
Is iron deficiency anemia microcytic or macrocytic?
Microcytic
What is a unique symptom of iron deficiency anemia?
what additional symptoms may be present?
Pica
Pallor (Skin, Conjunctiva)
Spoon Nails
Fatigue
Weakness
In a patient with iron deficiency anemia what would you expect the values to be (High or low) in a…..
TIBC?
MCV?
MCH?
Ferritin?
TIBC: High
Everything else is low
(Remember low MCV = microcytic, low MCH = hypochromic)
How is iron deficiency anemia treated?
When is this treatment given?
When are changes most likely to be seen?
How long until the anemia is corrected?
Iron Salts
Saccharated Iron
Given 30 minutes before meals
Changes are likely to be seen after 2 weeks of therapy
The anemia should be corrected in 2 months
If a patient fail to improve on the standard iron deficiency treatment, what may be occuring?
- Hemorrhage
- Infection / Cancer
- Insufficient iron intake
- Malabsorption
What is an additional cause low MCV?
Lead poisoning
_______ anemia is the premature breakdown of cells
Hemolytic anemia
_______ hemolytic anemia is due to an abnormal RBC cytoskeleton which produces accelerated RBC destruction in spleen
Intrinsic
___ _______ is X-linked and commonly presents following an acute illness or medication use and is more common in african american populations and in males.
G6PD Deficiency
G6PD Defficiency is ___-linked and common in _______ ________ that can result in hemolysis after acute ______ or intake of certain drugs that produce peroxide and causes oxidation of Hb and RBC membranes.
X-Linkes
African Americans
Illnesses
What medications are commonly associated with G6PD Deficiency?
(THINK: anything that damages RBCs)
- Antimalarials (Primaquine)
- Analgesics (Phenacetin, ASA)
- Sulfonamides
G6PD Deficiency is typically asymptomatic.
What symptoms may you notice if there was active hemolysis occurring?
Malaise
Lumbar Pain
Abdominal Pain
- Anemia
- Jaundice
- Dark Stool
(*Indicative of progressive disease)
Heinz bodies and bite cells seen on peripheral smear are indicative of what deficiency?
G6PD Deficiency
What is the most diagnostic lab value is diagnosing G6PD Deficiency?
G6PD Assay
High levels during hemolysis
How is G6PD Deficiency treated?
What should be avoided?
Avoid Triggers
Supportive Care
Rarely give transfusions
AVOID fava beans
What is the most concerning complication of G6PD Deficiency?
Hemoglobinuria
AKI
_____ ____ _____ is a normocyctic, normochromic anemia that is more prevalent in the African american population
Crisis in this disease is typically caused by infection.
Sickle cell anemia
2 parents with sickle cell trait have ____% chance of having a child with Hgb SS.
25%
What is the most common bacteria that causes crisis in sickle cell?
Most common cause of osteomyelitis?
- Pneumococcal
- Salmonella
- Staph Aureus
Sickle cell is autosomal _______.
Dominant
Sickle cell often results due to a defective ___-chain
Beta
What do pain crisises result from in sickle cell patients?
Sickled RBCs stick together and clog capillaries causing organ ischemia
What are some of the MANY symptoms associated with sickle cell anemia and crisis?
Anemia
Jaundice
Arthralgias
Fever
More severe Sx….
Poor-healing ulcers in the pretibial area Hemiplegia (CN Palsies) Pulmonary Dysfunction Renal Dysfunction Cardiomegaly Hepatosplenomegaly Cholelithiassis Aspetic femoral head necrosis
In a patient with sickle cell anemia what would you expect the lab values to be/show in a……
Reticulocyte count?
Peripheral smear?
CBC?
Elevated reticulocytes (especially in crisis)
Peripheral smear would show sickled ells with Howell-Jolly bodies
CBC may show a leukocytosis with left-shift
What is the most definitive diagnostic test for sickle cell anemia?
Positive Hgb S chains on hemoglobin electrophoresis
How is sickle cell anemia treated?
- High flow o2
- Pain control in crisis (Corticosteroids)
- Treat underlying infection
- Update vaccinations
What can be used to decrease the recurrence of crisis in sickle cell anemia patients?
Hydroxyurea
If osteomyelitis is diagnosed in a sickle cell anemia patient, how should this be treated?
- Fluoroquinolone x4-6 weeks with Rifampin
- Pnuemococcal vaccination
- Genetic counseling
- Prophylatic Abx (Penicillin in kids < 5 yo)
Transfusion should only be considered and is only supportive in sickle cell anemia if the hgb is ___.
< 6
What are two potentially fatal complications of sickle cell anemia and crisis?
Acute Chest Syndrome
Splenic Sequestration
__________ are in general the most common inherited hemoglobin production disorders
Thalassemias
___-thalessemia results from unbalanced Hb synthesis caused by dec production of at least 1 globin polypeptide chain (β, α, γ, δ) that result in abnormally shaped microcytic RBC that are prone to hemolysis causing anemia
Beta-thalessemia
Are thalessemias microcytic or macrocytic?
Microcytic
In Beta-thalessemias….
Are heterozygous typically symptomatic or asymptomatic?
Homozygous?
Heterzygous are typically asymptomatic
Homozygous are typically more severe and show bone marrow hyperactivity
Hgb in thalessemia can be normal at birth, why?
When would a patient become symptomatic?
The presence of fetal Hgb
6 months of age
What is a common symptom in thalessemia?
(Think: RBC Sequestration)
What additional symptoms may be seen?
Splenomegaly
- Delayed Puberty
- Jaundice
- Leg Ulcers
- Cholelithiasis
What shape would you expect to see cells on a blood smear of a thalessemia patient?
Teardrop shaped
In a patient with thalessemia……
What would the MCV be?
Reticulocyte count?
TIBC?
Ferritin?
RBC Count?
MCV: Low
Reticulocyte COunt: Low
TIBC: Normal
Ferritin: Normal
RBC Count: Normal to high
T/F: Thalessemia would look less microcytic and hypochromic than iron deficiency
False
It appears MORE microcytic/hypochromic
What is the life expectancy in a patient with beta-thalessemia Major?
minor?
Major: Many people do not live to puberty
Minor: Normal life expectancy
How could you treat beta-thalessemia major?
Minor?
Major:
Splenectomy (to decrease number of transfusions)
RBC transfusion (limit number to decrease iron overload)
Chelation Therapy
Rarely due stem cell transplant
Minor:
No Tx
What are three concerning complications of Beta-thalessemia?
- HF (from iron deposits)
- Cirrhosis
- iron Overload
What would be seen on electrophoresis and blood smear in a beta-thalessemia patient?
+ HB on electrophoresis
Smear: many nucleated erythroblasts, target cells, small RBCs, etc…
What is the most diagnostic test for Beta-thalessemia diagnosis?
Elevation of Hb A2
In alpha-thalessemia….
How does it effect heterzygotes?
Heterozygotes with 2 of 4 gene defects?
Defects in in 3 of 4 genes?
All 4 genes?
Heterzygotes: Clinically normal
2 of 4 genes: mild-moderate microcytic anemia without symptoms
3 of 4 genes: Impairs alpha chain production resulting in increase beta and gamma chains
All 4 genes: Lethal in utero
How is alpha-thalessemia diagnosed?
Normal HbF and Hb A2 with +HB on electrophoresis
Alpha-thalessemia is typically not treated but if severe anemia and splenomegaly occur what is an option?
Splenectomy
This form of anemia results from a history of iron deficiency, chronic infection, inflammation, cancer, RA, HIV, or Cirrhosis.
Anemia of chronic disease
Anemia of chornic disease can be either ________ or ________
Macrocytic or normocytic
_________, produced by the liver binds to ferropotin which ______ the exportation of iron into the blood.
Hepcidin
Inhibits
Renal insufficiency with a low serum EPO would be indicative of what?
Anemia of renal failure
In anemia of chronic disease…..
What would you expect the lab values to be for ferritin?
TIBC?
MCV?
Serum EPO?
Ferritin: High (Sometimes normal)
TIBC: Low
MCV: Low
Serum EPO: Low (in renal failure anemia)
How is anemia of chronic disease treated?
- Treat the underlying disorder
2. Recombinant EPO and iron supplements
Why should EPO analogs be stopped once Hgb gets above 11?
To reduce the risk of MI/CVA
_________ anemia commonly occurs in the young, can result as a side effect of drugs, and is the only anemia that effects all three cell lines resulting in pancytopenia.
aplastic
Aplastic anemia is __________ and normochromic
Normocytic
Aplastic anemia often results from loss of blood cell ______ and results in _________ of bone marrow, RBC, WBC’s and platelets.
This is anemia without ________
Precursors
Hypoplasia
Reticulocytes
What types of drugs/medications are associated with aplastic anemia?
What virus (especially in sickle cell patients)?
Drugs/Medications:
ACEi
Sulfonamides
Chemotherapy
Radiation
Virus:
Parvovirus B19
__________ anemia accounts for 25% of all aplastic anemia cases.
Fanconi
Sx of aplastic anemia vary depending on the severity of pancytopenia…..
what Sx would you expect to see?
What Sx would you see if the patient was thrombocytopenic?
What symptoms would not be present in an aplastic anemia patient?
Sx:
Fatigue
Weakness
Pallor
Pupura
Thrombocytopenic:
Petechiae
Ecchymosisi
Gingival Bleeding
Sx NOT Present:
Hepatosplenomegaly
Bone pain
Lymphadenopathy
In an aplastic anemia patient, what would you expect the lab values to be in a…..
CBC?
Serum Iron?
MCV?
Reticulocyte count?
CBC:
Low WBCs, RBCs, Platelets
Normocytic
Serum Iron: High
MCV: Normal
Reticulocyte count: Low
If an aplastic anemia patient’s lab showed a normocytic anemia, rectiulocytopenia, with normal WBCs and platelets…..
This patient is said to have pure _____ aplasia
RBC
How is aplastic anemia treated?
- Stop the offending agent
2. Transfusion
If an aplastic anemia patient severe enough, what can be given to increase the survival?
What additional treat option is available?
antithymocyte globulin [ATG] + cyclosporine
- If severe and > 40 y.o
- If severe and < 40 y.o. and no HLA-identical sibling donor
- If not severe but are transfusion-dependent
Bone marrow transplant is also an option
_____________ is a decrease in the number of platelets
Thrombocytopenia
What are THREE causes of thrombocytopenia?
- Decreased production (ie: leukemia)
- Increased destruction (ie: ITP)
- Medication induced (ie: HIT)
_________ ___________ ________ commonly occurs following a viral illness can be insidious and chronic in adults but acute and self-limiting in children and is regarded as an autoimmune process.
Idiopathic thrombocytopenic puupura (ITP)
Describe the pathphysiologic process of ITP
Autoimmune antibody reaction to platelets which results in splenic platelet destruction often after an acute infxn
What Sx are commonly seen in ITP?
What Sx is NOT seen in ITP?
Petechiae
Purpura
Mucosal Bleeding
NO splenomegaly
In a patient with ITP what would be the lab values in a…..
CBC?
CBC would show low platelets with the rest being normal
Other than a CBC what additional labs should be ordered in a ITP patient if you suspect underlying infection like Hepatitis or HIV?
Coags
LFTs
ITP is a diagnosis of __________
Exclussion
A ______ needs to be ordered in ITP patients to assess for the Philadelphia Chromosome or JAK2 Mutation
BMBx
How is ITP treated in children?
Adults?
Children:
Supportive Care
IVIG is refractory
Adults:
Prednisone
____________ ________ _________ typically results following a febrile illness and can be seen with multiorgan thrombsis
Thrombotic thrombocytopenic purpura (TTP)
What Sx are commonly seen with TTP?
What would be a key symptom if there was cardiac damage in a TTP patient?
Sx:
FEVER Thrombus Weakness Confusion N/V/D
Cardiac Damage would result in arrhythmias in a TTP patient
What would be seen on a blood smear of a TTP patient?
Schistocytes
In a TTP patient what would you expect the lab values to be in a…..
CBC?
Reticulocyte count
LDH?
Bilirubin?
Comb’s Test?
CBC:
Low platelets
Anemia
Reticulocyte Count: High
LDH: High
Bilirubin: High
Comb’s Test: Negative (negative direct antiglobulin)
How is TTP treated?
How often?
What can be added in adults?
Plasmaphoresis
This occurs daily until platelet count normalizes
Corticosteroids can be added in adults
T/F: Kidney failure can be seen in patients with TTP
False
It is NOT seen
________ _____ ______ is similar to TTP but is more common in children and involves renal failure.
It can also be seen in patients with a Hx of E. coli infections and recent diarrheal illnesses.
Hemolytic Uremic Syndrome (HUS)
Is HUS considered autoimmune?
No
What deficiency is associated with HUS?
ADAMTS13 Deficiency
What Sx are seen in a patient with HUS?
DIARRHEA
Fever
Nuerologic Sx
Renal Failure
Microangiopathic Anemia
Thrombocytopenia
What lab would be noticeably low in a HUS patient?
Other than renal function
Platelet count
How is HUS treated?
How often?
What is given to reverse renal failure?
Plasmapheresis [plasma exchange]
Daily until platelet normalizes
Eculizumab
____ ________ is the most common genetic bleeding disorder and s autosomal dominant/
It is described as a missing protein important to platelet function and limits their ability to adhere to a vessel wall at the site of injury.
von Willebrand’s (vWB)
What types of things can improve clotting because they stimulate production of vWF?
Hormonal Changes Stress Pregnancy Inflammation Infection
What Sx are associated with vWB?
Excessive bleeding (cuts, surgery, menorrhagia)
Superficial Bleeding
Petechiae
LACK of hemarthosis
In a patient with vWB what would you expect the lab values to be in a……
vWF?
PT?
aPTT?
Platelets?
vWF: Low (Factor VIII may be low too)
PT: Normal
aPTT: Normal
Platelets: Normal
How is vWB treated?
Most do not need treatment
What can be given to a vWB patient about to undergo surgery who DOES NOT want transfusion?
DDAVP (Desmoporesis)
What can be given to vWB patients if excessive bleeding is present?
Intermediate-purity Favtor VIII
What can be used in women with vWB who are experiencing menorrhagia?
OCPs
IUD
________ is more common in males and is X-linked recessive
Hemophilia
Hemophilia __ is the most common and involves Factor VIII
Hemophilia __ involves Factor IX and is also referred to as Christmas Disease
A
B
What is the most common Sx seen in patients with hemophilia?
Hemarthosis (most commonly in the knee)
In a patient with hemophilia, what would you expect the lab values to be in a…..
PTT?
Factor VIII or IX?
Platelets?
PTT: High
Factor VII or IX: Low
Platelets: Normal
How is mild hemophilia treated?
severe?
What should be avoided?
Mild:
Aminocaproic acid / desmopressin
Severe:
Transfusion (Cryoprecipate)
Replace Factor
AVOID:
NSAIDs
ASA
What is a concerning complication of hemophilia?
Development of antibodies to transfused clotting factors
What are THREE ways a patient could have a hypercoaguable state?
(Not diseases)
- Problems with platelets (HIT)
- Vascular Injury
- Clotting Factors
_______-________ ________ typically occurs in 1% of patients after the administration of unfractionated heparin.
Platelet counts typically drop within 7-10 days following exposure
Heparin-Induced Thrombocytopenia (HIT)
_____________ HIT results in global thrombocytopenia and thromboembolism due to immune reaction with platelet factor 4²
Immunologic
Describe how HIT could result in MI or CVA
Platelets clump excessively and obstruct vessels leading to venous and arterial thromboses
LETHAL
T/F: Bleeding is commonly seen in patients with HIT
False
Rarely seen
What lab tests are used to diagnosis HIT?
Would the the patient be thrombocytopenic?
- ELISA followed by platelet assay confirm
Yes
How is HIT treated?
Other than heparin, what anticoagulant should be avoided until the platelet count improves?
- Stop heparin
- Antibody testing to platelet factor 4
- Anticoagulation (obviously without heparin)
AVOID:
Warfarin (Can give after platelet count improves)
______________ ___________ occurs in patients with a Hx of SLE an is referred to as a autoimmune disorder.
This results in thrombosis and (in pregnancy) fetal demise caused by antibodies acting against phospholipid binding proteins
Antiphospholipid Syndrome
In a patient with antiphospholipid syndrome, what would you expect a aPTT to be?
Elevated
T/F: A lupus anticoagulant is present in a patient with antiphospholipid syndrome
True
How is antiphospholipid syndrome treated?
Anticoagualtion:
Heparin
Warfarin
ASA
Prophylatically and for Tx
_____ ___ ______ ______ is the most common genetic hypercoaguable state and often is seen in younger patients with recurrent thromboembolic events without the presence of risk factors.
Factor V Leiden Mutation
What is the pathophysiology of Factor V Leiden?
Factor V is resistant to normal inactivation by protein C
What is a common Sx in patients with Factor V Leiden?
Venous thrombosis
What is the most definitive test for diagnosing Factor V Leiden?
What other test can be done?
Definitive:
Genetic testing for Factor V gene
Other:
Activated Protein C resistance Assay
How is venous thrombosis treated in a patient with Factor V Leiden?
What is given prophlyatically?
Parenteral Heparin OR LMWH followed by oral Warfarin
Prohpylaxis: Warfarin
______________ ____ is common in males 60 or older and commonly is associated with erythrocytosis involving a JAK2 mutation
Polycythemia Vera
What are two common symptoms seen in patients with polycythemia vera?
What additional symptoms may be seen?
Hepatosplenomegaly
Erythromelagia (LE erythema, warmth, pain, distal infarction)
Other:
Vertigo Tinnitus HA Visual Changes TIA HTN Hepatic Vein Thrombosis
in a patient with polycythemia vera, what would you expect the lab values to be in a…..
Hgb?
Hematocrit?
WBC?
EPO?
Hgb: >20
Hct: > 60%
WBC: High
EPO: Low to unmeasurable
Why would you order an abdominal US in a patient with Polycythemia Vera?
To assess for splenomegaly
What is the most definitive test for diagnosis of polycythemia vera?
Assay for JKA2 V617F
How is polycythemia vera treated?
What else can be done to reduce RBC mass and expand plasma volume?
Hydroxyurea
Phlebotomy
What TWO complications are associated with polycythemia vera?
Increased incidence of PUD associated with H plyori
Increased incidence of nonlymphocytic leukemia
_______ polycythemia is often associated with tissue hypoxia, abnormal EPO production, and peptic ulcer disease.
Secondary polycythemia
In a secondary polycythemia patient…..
Increased levels of EPO with a normal o2 saturation and a non-smoker what may be abnormal?
- Abnormal Hgb not delivery O2 to tissues
2. Ectopic source of EPO production not responding to normal feedback loops
Sx of secondary polycythemia are similar to polycythemia vera (ie: splenomegaly)….
but what additional symptoms may be appreciated?
Ruddy Complex
Easy Bruising
Epistaxis
GI Bleed
Hypoxia Sx (Cyanosis, HA, fatigue, impaired mental status)
T/F: In a patient with secondary polycythemia, Hgb will be > 20 and Hct will be > 60%
True
In a patient with secondary polycythemia….
What will assess for increased RBC mass?
Isotope dilution via Cr-labeled autologous RBC & sample blood radioactivity over 2 hrs
In a patient with secondary polycythemia….
If the RBC mass is normal what may be present?
Spurious / Relative polycythemia
In a patient with secondary polycythemia….
If RBC mass is increased what should be assessed?
EPO levels
What mutation should you always check for in a patient with secondary polycythemia?
JAK2
In a patient with secondary polycythemia….
if the arterial O2 is < 92% what should you assess for?
Heart and lung disease if not living in a hypoxic environment
In a patient with secondary polycythemia who is also a SMOKER….
What lab value with be elevated?
What does this diagnose?
Elevated COHb level
This diagnoses “Smoker’s polycythemia”
In a patient with secondary polycythemia….
A CT Abdomen/Pelvis is great for assessing what?
EPO producing neoplasms
In a patient with secondary polycythemia….
If a patient has elevated O2-Hgb affinity, what does this diagnose?
Abnormal Hgb
What is a concerning complication of secondary polycythemia and hypoxia
Cor Pulmonale
