Hematology / Oncology Flashcards
True or false: you should order antiplatelet antibody testing to evaluate ITP.
False
While there is such a test, it has low sensitivity and is not available at many hospitals. As such, ITP is still a diagnosis of exclusion. You must exclude the following things:
- Infections: HIV, HCV, HBV, H. pylori
- Autoimmune: SLE, vasculitides
- Nutrient deficiencies: B12, folate
- Malignancy: MDS, leukemia
If a patient has a thrombocytopenia-like bleeding pattern (e.g., epistaxis, gum bleeding, and petechiae) but there platelet levels are normal, you should consider ordering a ___________________.
platelet function analyzer 100
This test passes anticoagulated platelets through a collagen network and measures the speed at which the filter clogs up. Prolonged closure time indicates platelet dysfunction.
What is paraprotein?
A type of protein that inhibits clotting factors
True or false: all DOACs can be started without heparin bridging.
False
Apixaban and rivaroxaban can be started without bridging.
Dabigatran and edoxaban require heparin bridging.
Describe the utility of the pulmonary embolism severity index.
PESI is a score that predicts the 30-day mortality of pulmonary emboli using several criteria (age, sex, cancer status, CHF status, AMS, and vitals among other things). Those with PESI < 65 have a near 0% risk of 30-day mortality and can be managed outpatient. Those 65-85 have a near 1% risk and can go either way for disposition. Greater than 85 is considered higher risk and should be managed inpatient initially.
What is the mechanism behind inflammatory anemia?
IL-6 leads to proteolysis of the membrane protein ferroportin which is the protein that absorbs iron from the gut, thus lowering the serum iron without affecting iron stores already within cells.
Inflammatory anemia usually has what iron panel profile?
High ferritin
Low TIBC
Low serum iron
Discuss the different treatment options for MDS based on risk status.
In patients with high risk disease (which is determined by genetic analysis), allogenic stem cell transplantation is pursued if the patient is young and healthy enough to tolerate it. In patients with low-risk disease, therapy is aimed at limiting the need for transfusions. Specifically, lenalidomide can help delay progression and limit transfusion need.
Lenalidomide works by _______________.
affecting both the humoral and cellular immune system
Describe hypereosinophilic syndromes.
High levels of eosinophils (>1.5) can cause organ damage regardless of why they are elevated. The most common organs involved are the skin (eczema, erythroderma, urticaria, and angioedema), lungs (parenchymal infiltrates, pleural effusion, lymphadenopathy, and pulmonary emboli), gastrointestinal tract (eosinophilic gastritis, enteritis, colitis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, Budd-Chiari syndrome), and heart (mitral or tricuspid regurgitation, cardiomegaly, restrictive cardiomyopathy).
Describe the management of anemia in people with hereditary spherocytosis.
Those with HS usually just have a mild anemia that warrants no intervention. There are some instances where they do need interventions, though:
- Chronic severe hemolytic anemia: rarely, those with HS will have more severe anemia and require splenectomy to limit hemolysis.
- Aplastic anemia: those with HS are at increased risk of virally induced aplastic anemia and can require transfusion.
Prednisone treats what kind of anemia?
AHA
D-dimer is only useful in _____________________.
evaluating those with a low probability of PE
It isn’t perfectly sensitive, so if someone has a high probability of PE then you would need to do additional imaging regardless of what the D-dimer is.
How should you diagnose PE in pregnant women?
- First, do PVLs. If they have DVTs you can presume their pulmonary symptoms are from PE.
- Second, if PVLs are negative do a V/Q scan.
- Third, only do a CTA if the V/Q is equivocal.
Why is it important to deal with anemia preoperatively?
Preoperative anemia has been associated with increased perioperative mortality, particularly when it is IDA. Addressing iron deficiency before a surgery can boost survival and limit intervention.
True or false: the preferred agent (per the boards) to reverse warfarin toxicity urgently is FFP.
False
It is Kcentra (4-factor prothrombin complex concentrate). This is preferred because it is quicker to give than FFP and saves volume for those in whom volume is a concern. Of note, Kcentra costs $5k per dose.
It used to be that guidelines recommended searching for _____________ in those with unprovoked DVT.
malignancy
Studies have shown mixed results in looking for occult malignancy, so currently guidelines recommend only being up to date on standard cancer screens like Pap smears, mammograms, and colonoscopies.
B2 microglobulin is used to risk stratify ______________.
MM
Patients with multiple myeloma will sometimes get ppx ______________.
IVIG (because of hypogammaglobulinemia)
A patient who has evidence of hemolysis (e.g., elevated indirect bilirubin and low hgb) as well as low-grade fever a few days after receiving a blood transfusion may have _______________.
delayed hemolytic transfusion reaction
In this syndrome, a person has low-level antibodies to a non-ABO antigen on RBCs (say, from a past transfusion or pregnancy). When they get blood that may have that antigen, they won’t hemolyze as aggressively as frank ABO mismatch, but slowly the will lyze the cells and may present days later like the stem described.
__________________ should be supplemented in DIC to limit bleeding.
Cryoprecipitate
True or false: most adult Parvovirus infections entail arthralgias and rash.
False
Most are actually asymptomatic, which is why you need to have a high index of suspicion in a person presenting in aplastic crisis.
True or false: aplastic anemia most commonly presents with pure red cell aplasia.
False
Aplastic anemia most commonly involves all cell lines.
When should essential thrombocytosis be treated?
In patients > 60 years old who have had thromboembolic events.
Treat with hydroxyurea.
List some causes of secondary thrombocytosis.
- Iron deficiency
- JAK2 kinase mutations
- Malignancy
- Bleeding
- Collagen vascular diseases
Benign neutropenia is a condition in which ______________________.
ethnic minorities can have persistent neutropenia to 1.0-1.5 range; it is not associated with increased risk of infection
G6PD levels should not be measured _____________.
during an acute hemolytic crisis; it will be falsely normal because the newly made RBCs will raise G6PD serum levels even when the absolute levels are low
How can the iron panel help differentiate anemia of kidney disease vs inflammation?
In inflammation, serum iron and TIBC is low whereas in CKD it is usually normal.
______________ is diagnosed by abdominal fat pad biopsy.
Amyloidosis
Review the presentation of Waldenstrom macroglobulinemia.
WM is an indolent B-cell lymphoma that presents with B symptoms, anemia, hepatosplenomegaly, and an elevated IgM spike. Bone marrow biopsy is needed to confirm the diagnosis.
Atypical presentations of rheumatoid arthritis might be _______________.
hemochromatosis
Particularly look out for the “hook-like osteophytes” of the 2nd and 3rd MCP joints.
The best initial test for hemochromatosis are _________________.
transferrin and ferritin
High transferrin and high ferritin are classic. Normal of both has a good negative predictive value.
When is tPA indicated in the treatment of DVTs?
When limb ischemia is suspected (e.g., absent pulses, cyanosis, etc.)
In what situation can you give blood that doesn’t crossmatch?
In severe autoimmune hemolytic anemia, even correctly ABO-matched blood will often fail to crossmatch. If a person is having anemia-related symptoms or is severely anemic, you should give them blood that fails to crossmatch because it will still survive for several days during which you can immunosuppress them.
True or false: the most common problem after autologous bone marrow transplant is GVHD.
False
With autologous transplants you do not get GVHD. The most common complication is infection.
What workup is needed when a polyclonal M spike is noted?
- BMP: renal function, Ca
- CBC: anemia
- Whole body MRI: looking for lytic lesions (more sensitive than x-ray)
At which level should you consider iron removal therapy?
Ferritin > 1000 in a patient with no concern for inflammatory elevation of ferritin
Use deferoxamine (IV) or deferasirox (PO)
What hgb target is usually used when giving EPO agents to those with anemia of CKD?
11-12
This helps to limit some of the risks of EPO (HTN, strokes).
______________ is the reversing agent for dabigatran.
Idarucizumab
Those with sickle cell are at increased risk of perioperative complications. A landmark trial in 1994 showed that ___________________ helped to decrease the risk substantially.
simple transfusion to >10
What thrombophilia is associated with an increased risk for warfarin-induced skin necrosis?
Protein C deficiency
Remember that warfarin-induced skin necrosis is caused by a transient hypercoagulable state when proteins C and S, which are both anticoagulant factors, are knocked out by warfin before the procoagulant factors 10, 9, 7, znd 2. Those with protein C deficiency are at increased risk because they already have low levels of C.
Side note, protein C deficiency is an autosomal dominant disorder. One missing copy leads to 50% of the level of protein C and thus the increased risk. (I’m guessing a homozygous mutation would be fatal because you would not be able to anticoagulate at all, but I’m not sure.)
Describe the utility of the PE screening scores.
• PERC (PE Rule-out Criteria): a series of questions designed to screen-out PE. If all answers are no, then the probability of PE is so low that no imaging or further workup are needed. If any are positive, then the clinician should consider imaging.
• Wells: This stratifies people into low-, medium-, and high-risk categories. Low-risk patients can be screened out with no further testing. Medium-risk patients should get a D-dimer; if the D-dimer is negative then no further testing need be done. Lastly, high-risk patients should go straight to imaging because a negative DD does not rule-out PE in high-risk pts.
Paroxysmal nocturnal hematuria leads to low levels of which cell lines?
RBCs, WBCs, and platelets
This happens because the CD55 and CD59 receptor that prevents the activation of complement is present on all blood cells.
If a patient has clear hemolyis by their labs but a completely normal smear (i.e., no spherocytes, schistocytes), then think ____________.
PNH
In PNH, you have transient hemolysis so the smear can normalize but the labs won’t.
Diagnose PNH with ______________.
flow cytometry
Coagulopathy of liver disease can look like DIC. What lab can distinguish the two?
Factor VIII level
Because VIII is produced in endothelial cells, it is not affected by the liver. Moreover, it will often be high in liver disease because the body senses the hypocoagulable state.
Those with sickle cell anemia can have baseline hgb in the _______ range.
5-7
Review the types of alpha thalassemia by genotype.
Remember that there are four alpha hemoglobin genes:
•One gene broken: silent carrier, no symptoms
• Two genes broken: alpha thal trait, mild anemia
•Three genes broken: hemoglobin H disease, severe, transfusion-dependent anemia
• Four genes broken: hydrops fetalis
Increased levels of A2 is a sign of __________.
beta-thalassemia minor (Barts)
Review the INR ranges and their interventions.
*Elevated INR and any major bleeding: give prothrombin complex concentrate
*INR 3-10 and no bleeding: withhold warfarin and recheck
*INR > 10 and no bleeding: withhold warfarin and give vitamin K
Also, anticipated surgical procedures can also indicate vitamin K.
True or false: TTP-HUS in a pregnant woman typically presents with schistocytes.
False
_____________ is the treatment for atypical HUS.
Eculizumab
True or false: those with SS disease will have about 60% HgB A.
False
Those with SS form of SCD will have no hemoglobin A.
Those with sickle trait can have pain crises if ______________.
They also have beta minor.
What are indications for hydroyxurea therapy in those with SCD?
Frequent pain crises or vaso-occlusive events like ACS and CVA
Hydroxyurea increases fetal hemoglobin and decreases vasospasm and platelet activation, all of which decrease the likelihood of vaso-occluisive events
True or false: ADAMTS13 is needed to confirm the diagnosis of TTP.
False
That test can take a while and TTP has such a high mortality (90% if untreated) that you need to begin treatment sooner than that. When you have clinical findings (fever, AMS) and lab findings (hemolytic anemia, AKI) with no concern for competing diagnoses like HUS or DIC, then start PLEX.
Describe the diagnosis of ACS in those with SCD.
It is essentially like PNA that evolves quickly: fever, respiratory distress, infiltrates on imaging, and rapid progression. You treat it like PNA (abx, fluids, O2 support), then when it progresses you intubate and do exchange transfusion.
Review the management of thromboembolism as presented in Moll’s article “How I Treat: Unexplained Arterial Thrombosis.”
1) First, clearly define the clot. Obtain whatever imaging you need to in order to know where it is (i.e., venous or arterial), how big it is, and what watershed areas it is affecting.
2) When you know the areas that it is affecting, then consider a consultation to that organ’s specialist (e.g., if it is a hepatic thrombosis, then consult a hepatologist to evaluate the extent of liver damage.).
3) Review for the causes and risk factors for thromboembolism:
- Echo (PFO, aneurysm, valvular problems)
- Carotid Dopplers (if cerebral)
- EKG and telemetry for pAF or other arrhythmia
- Imaging for local atherosclerosis (could be CTA, MRA, or US Doppler)
- Risk of atherosclerosis in history (immobility, obesity, HTN, tobacco use, dyslipidemia, DM, cocaine, amphetamine) or FMH
- Review medications for pro-thrombotic agents (most common ones are OCPs, HRT, anabolic steroids, recent heparin use, and IVIG)
- Look to see if they are up-to-date on malignancy screening (or have signs/symptoms that could potentially be malignancy)
- Consider evaluation for vasculitis or other inflammatory disorders
- Consider imaging the organ in question for anatomic anomalies that could predispose to clot formation (like fibromuscular dysplasia)
- If the above does not illustrate a likely cause, consider a thrombophilia workup — particularly if the patient is young (see the other card)
What malignancy screening is warranted after an unexplained VTE?
Age-related screening, risk-factor-related screening, and symptom-guided screening only
The big picture is don’t go hunting for cancer if the person is otherwise asymptomatic and up-to-date on their screenings (e.g., don’t do PETs on people just because of a clot).
Once you have followed the thorough initial workup of arterial thrombosis and found no secondary cause, what DDx and workup should you consider?
- Primary thrombophilias (FVL, PT 20210, PC, PS, AT, homocystinuria)
- Antiphospholipid syndrome
- Paroxysmal nocturnal hemoglobinuria
- Hyperviscosity syndromes (Waldenstrom, MM, erythrocytosis, thrombocytosis)
- Myeloproliferative neoplasms
- Vasculitis (particularly Behcets, Takayasu, giant cell)
- Sarcoidosis
Review the testing for APLAS.
The diagnosis requires the clinical history of venous or arterial thromboembolism as well as miscarriage in women and lab testing as below:
- Lupus anticoagulant, anti-cardiolipin IgG and IgM, and anti-beta2-glycoprotein 1
- Two sets of the above at least 12 weeks apart
Note: the above antibodies can be positive in the setting of inflammation so be cautious with positive results in someone with signs of inflammation.
What does research suggest about plasminogen mutations in clot risk?
There is no definitive research to suggest that plasminogen and plasminogen activator mutations have clinical syndromes associated with clotting.
What do guidelines say about managing arterial thrombosis in APS?
Trick question, no consensus exists.
Aspirin alone, anticoagulation alone, and combination are all frequently used. There is some data that suggests that warfarin leads to improved outcomes over DOACs and ASA, so you may hear that warfarin is the right choice, but it is far from a complete picture.
How does management of an arterial thrombosis change if a thrombophilia is diagnosed?
All people who have had an arterial thrombosis should be on lifelong baby aspirin because of the documented decrease in risk of arterial thrombosis and their low risk of bleeding. There is some data suggesting that aspirin and anticoagulation decreases the risk of arterial clots in those with a thrombophilia, but the risk of bleeding is higher. Because of this, those with a thrombophilia should have a discussion about the risks and benefits of dual therapy.
One unit of platelets should raise your counts by ____________.
30-50
Review the types of transfusion reactions.
•Febrile non-hemolytic: presents with fever only; is a rule-out diagnosis (meaning you need to assess for acute hemolytic reaction and septic transfusion)
•Hemolytic: presents with hemolysis, flank pain, hypotension; can be delayed or acute
•Allergic: presents with itching and hives
•Septic: presents with fever, rigors, bacteremia
•TRALI: presents with acute respiratory distress that does not respond to diuresis
• TACO: presents with volume overload
What should you do to assess for hemolytic reaction in a person with a fever post-transfusion?
• Get vitals (looking specifically for hypotension)
•UA (look for hemoglobinuria)
•Assess for flank pain
Recurrent allergic reactions to transfusions should prompt evaluation for ______________.
IgA deficiency
List five risks five potential complications of massive transfusion protocol (MTP).
•Coagulopathy (the anticoagulants in blood products can prolong PT)
•Hypocalcemia (the chelating agents in blood products do this)
•TACO
•Hyperkalemia
•Hypothermia (from blood being stored cold)
List the percentage of factor activity that correlate with mild, moderate, and severe hemophilia.
•Mild 5-20%
•Moderate: 1-5%
•Severe: <1%
What should you evaluate for if a hemophiliac who has gotten lots of exogenous factor suddenly stops responding to that factor?
Acquired factor inhibitors
Review the types of von Willebrand factor deficiency.
• Type 1: quantitative defect, leads to concordantly low vWF activity and antigen; caused by genetically low levels of vWF
•Type 2: qualitative defect, leads to low activity with normal antigen; caused by inhibitors
Severe megaloblastic anemia can actually cause ___________.
hemolysis (from the enlarged cells being unable to exit the marrow)
Describe the presentation of myelodysplastic syndrome.
•Pancytopenia
•Macrocytosis
•Dysplastic cells on smear (hypogranular neutrophils, messed up segmentation, nucleated erythrocytes)
To diagnose it, you must rule out other causes of pancytopenia and then get a bone marrow biopsy.
Those with SCD are at increased risk of developing what chronic cardiopulmonary condition?
Pulmonary hypertension (particularly pregnant women)
The ________ score helps predict the risk of HIT.
4T
What makes HUS atypical?
When it is not preceded by a diarrheal illness
Patients with lytic lesions from multiple myeloma should be given _____________.
zoledronic acid
Positive antibodies to C3 are found in ____________.
cold agglutinin syndrome
______________ can prevent the aPTT from rising when heparin is given.
Antithrombin deficiency
Those with autoantibodies against a factor will need __________________.
infusions of other factors that circumvent that pathway; for instance, a factor VIII autoantibody syndrome can be treated with factor VII
Approximately 50% of cases of Budd-Chiari are seen in patients with what hematologic problem?
Myeloproliferative neoplasm
This is most often seen in patients with erythrocytosis or thrombocytosis, but it can be seen in patients without CBC abnormalities. As such, consider JAK2 testing and smear review in all people with abdominal thromboses.
In addition to regular hemolytic anemia, _____________ can also present with a positive DAT.
drug-induced hemolytic anemia
Those who have had _____________ are more likely to develop symptomatic babesiosis from transfusions.
splenectomy
Remember the presenting symptoms: fever, transaminitis, thrombocytopenia, and hemolytic anemia a couple weeks after a transfusion.
Those with chronic ITP are usually just monitored if their platelets are above ______.
30
What are the two subtypes of TTP?
Hereditary and acquired
Hereditary TTP results from a genetic mutation in the gene that encodes the ADAMTS13 protease.
Acquired TTP results from auto-antibodies against the protease.
The ____________ score is used to quantify the likelihood of TTP.
PLASMIC
This is a scoring system from 0 to 7. In a nutshell, you get high points for having thrombocytopenia and hemolytic anemia in the absence of AKI, cancer, and coagulopathy.
To diagnose someone with TTP, you generally start with a PLASMIC score to gauge likelihood while you await the ADAMTS13 to confirm. You can start treatment in PLASMIC >4.
If the ADAMTS13 level is greater than ____%, then the diagnosis of TTP is unlikely.
20
Less than 10% is highly suggestive (though not totally confirmatory as there may be other reasons why it is low and a person has a MAHA).
When should you put a VTE/PE patient on heparin?
When you anticipate a procedure or a patient is morbidly patient
There is a benefit to doing LMWH or DOACs because the patient is immediately therapeutic, whereas in heparin gtt you are chasing a PTT.
Any B12 level less than _________ should be treated.
300
What are the three big categories of pancytopenia?
Marrow replaced:
- Cancer (Mets or primary)
- Not cancer (infection, myelofibrosis)
Marrow failure:
- Destroyed (drugs, radiation, aplastic anemia, HLH)
- Deficiency (nutritional [B12, folate, Cu, Zinc] alcohol)
Peripheral:
- Consumptive (MAHA, DIC)
- Sequestration (cirrhosis, splenomegaly)
What test can help evaluate hemolytic anemia?
Reticulocyte count
Think of this, particularly if someone has a normocytic anemia.
What infections can lead to cold AIHA?
Mycoplasma
EBV
CMV
True or false: a negative Coombs rules out AIHA.
False
Some AIHAs only test positive on the “super Coombs” test.
The first like treatment for warm AIHA is __________.
steroids
How do you differentiate bone pain from malignancy vs growing pains?
Growing pains usually happen at night and don’t interfere with activities. Malignancy usually causes severe pains at any time of day.
Why is it that those with ITP have less bleeding than those with the same platelet count from other etiologies?
ITP causes large platelets that get counter as low but are functionally large.
What is the key question you need to ask in a child with isolated thrombocytopenia?
Is there active bleeding?
If a child is well appearing, does not have b symptoms, and I’m has a reassuring physical exam then even severe thrombocytopenia warrants only observation.
Review the four types of immune monoclonal antibodies.
• Naked antibodies: these simply bind to markers on tumor cells (opsonizing). They rely on the bodies immune system to find the labeled tumor cells. Example dinutuximab for GN2).
• Chemo-bound antibodies: chemotherapy bound to an antibody that gets absorbed into the cell (like gentuzumab).
• Bite / DART: two-faced antibodies where one face binds the cancer cell and the other binds T cells.
• CAR-T: engineered T cells that attack cancer.
What is a normal absolute reticulocyte count?
~55 x 10^9 cells/L
Review the emergent diagnoses that can happen to someone with sickle cell.
- Stroke
- Acute chest syndrome
- Splenic sequestration
- Sepsis (they are predisposed to bacteremia, particularly with Streptococcus pneumoniae)
- Veno-occlusive crisis (pain crisis, dactylitis, priapism)
- Osteomyelitis
- Aplastic crisis
Review the nomenclature of sickle cell.
- SS = homozygous for sickle beta hemoglobin
- SC = one beta sickle, one beta C
Review what you see on a newborn screen for sickle cell.
- FS = sickle cell (because you have fetal hemoglobin which is alpha/gamma and sickle hemoglobin which is alpha/beta)
- FAS = sickle trait (same as the above but you also have regular alpha/beta adult hemoglobin)
- FCS = sickle c disease
What lab sign can tell you a person with SS is about to have splenic sequestration?
Low platelets.
It’s thought that the platelets get consumed in the spleen and lead to the engorgement of the spleen with RBCs.
Why should you be careful how much you transfuse a person with splenic sequestration?
Giving normal (non-sickle) blood to a person with splenic sequestration will cause their vaso-occlusive symptoms to go away, which will release all of the blood that the spleen has sucked up. Giving a transfusion can thus lead to auto-transfusion which can dramatically raise their hemoglobin. If their hemoglobin rises too high then they might have a CVA.
How many pediatric cancer cases are there per year?
15k
When you are considering a new cancer diagnosis or a cancer complication, what are the things that are emergencies? What could kill someone soon?
- TLS
- Airway compression (from a tumor or mediastinal mass)
- SVC syndrome
- Clots
- Bleeds
- Infections
TRALI occurs how long after transfusion?
Usually within 6 hours
How do you reverse dabigatran?
Idarucizumab
What is the reversal agent for DOACs?
Kaycentra (PCC or prothrombin complex concentrate)
PCC contains what factors?
X, IX, VII, II, C, and S
When a patient with hemophilia presents with a bleed, you want their factor level to be ___________.
100% for a major bleed or hip bleed and 50% for hemarthrosis to non-hip joints
What doses of factor do you need for bleeds in hemophilia A and B?
A: 50 units/kg of factor VIII
B: 100 units/kg of factor IX
Check to see if the patient has an inhibitor, because if they do then you need to give higher doses.
Presence of _____________ in CLL indicates poor prognosis.
anemia or thrombocytopenia
What is the management algorithm for pediatric ITP?
If children have no bleeding and platelets above 30,000 then observation is recommended because they are likely to get better without treatment. If bleeding is present (which it often is because that is the usual method by which kids present) then offer IVIG.
Review the management of LCIS.
Lobular carcinoma in situ (LCIS) is not a premalignant condition. However, if it is discovered on core biopsy then an excisional biopsy is recommended to confirm that it is LCIS and not DCIS. Some LCIS gets upgraded to DCIS.
Lobular carcinoma treatment beyond excisional biopsy is controversial. Some oncologists treat with SERMs and others don’t.
What anticoagulation should you give in suspected heparin-induced thrombocytopenia?
Start a non-heparin analog such as argatroban or bivalirudin and then transition to warfarin when the platelet count rises to 150k or greater.
What is the big difference in ITP in children vs adults (hint, it’s a timeline/course of illness issue)?
It is a self-limiting disorder in children, whereas in adults it’s a chronic condition.
When should you give steroids to an adult with ITP?
When platelets are less than 30k or with bleeding
The main risk factor for mesothelioma is _____________.
asbestos exposure
Febrile non-hemolytic transfusion reactions happen because of _____________.
residual leukocytes
Leukoreduction is indicated if someone has had a past FNHR.
What is typical blood volume in ml/kg for adults?
70 ml/kg
Review the three mechanisms behind leukostasis.
- Hyperviscosity from severe leukocytosis
- Tissue hypoxia from hypermetabolic leukocytes
- Vasomotor dysfunction from cytokines
What percentage of high-risk patients with a negative D-dimer have a PE?
Greater than 5%
What is the mechanism of the treatment for methemoglobinemia?
Methemoglobinemia is a condition in which the iron in hgb is oxidized to 3+. The treatment (methylene blue) reduces it to 2+.
Review the absolute contraindications to tPA.
- Known cerebrovascular malformation
- Known CNS neoplasm
- Ischemic stroke within three months
- Active bleeding (excluding menses)
- Recent head/face trauma
- H/o intracranial hemorrhage
Note: what the “absolute” part means depends on the case. If a person is coding from a known PE and you cannot get ROSC, for instance, then tPA is reasonable in some of the above cases.
In which patients do you need to go straight to Doppler US to rule out DVT?
Those with high pretest probability
If someone has low or moderate risk (based on Wells DVT criteria) then you can order a D-dimer first.
UNC’s protocol for ICH is to give KCentra for ______________..
all patients with ICH
The dose is based on INR. If INR is unknown then give a standard dose.
Four-factor prothrombin complex concentrate (PCC) is also called _________.
KCentra
The reversal agent for apixaban and rivaroxaban is __________.
Andexxa
Plasmapheresis is successful in what percent of cases of TTP?
80%
Those with acute chest syndrome should be treated with blood transfusion for a hgb goal greater than what?
10
In what non-deep venous thromboembolisms should you consider treatment?
Calf veins
Studies show that about 1/3 of calf veins (such as the tibial vein) progress to embolization or DVT. As such, symptomatic patients or those with risk factors should be considered for anticoagulation unless they are high risk for bleeding.
Review the treatment of ITP by the following presentations:
- No to mild bleeding:
- Moderate to severe bleeding:
- Severe, life-threatening bleeding:
- No to mild bleeding: corticosteroids
- Moderate to severe bleeding: corticosteroids, IVIG
- Severe, life-threatening bleeding: corticosteroids, IVIG, and platelet transfusion
What is the universal donor for FFP?
AB
Unlike pRBCs, FFP contains antibodies. Thus, type O blood is the worst donor because it contains all the antibodies.
What is the triad of Horner syndrome?
Ipsilateral miosis, ptosis, and anhidrosis
Which medicine lowers uric acid levels by stimulating the breakdown of uric acid?
Rasburicase
Rasburicase stimulates urate oxidase which breaks down uric acid.
Allopurinol inhibits xanthine oxidase which limits the production of uric acid.
Which medicine is first-line for treating hyperuricemia in patents with TLS?
Rasburicase
True or false: Kcentra can be used to treat hemophilia A.
False
Hemophilia A means no factor VIII. Kcentra is a four-factor concentrate of factors X, IX, VII, and II (“1972”).
It could treat hemophilia B, which results from factor IX deficiency.
Giving tPA will make your labs look like what syndromes?
DIC
The breakdown of clot releases D-dimer and consumes fibrinogen.
How should you manage major bleeding after tPA?
10 units of cryoprecipitate
Can also consider TXA/amicar, platelets, and PCC.
Splenic sequestration is also called _________________________.
hypersplenism
Splenosis is _______________.
seeding of the peritoneal fluid with spleen cells
The nadir of ANC happens when?
7-10 days after chemo
How does estrogen increase clot risk?
It induces increased production of the coagulation factors by the liver.
What are risk factors for developing TTP?
Pregnancy
AIDS
Autoimmune conditions (SLE, scleroderma, Sjögren’s)
Drugs: cyclosporine, tacrolimus, quinidine
What range of platelets is typical of TTP?
10-50
What is the pathophysiology of febrile non-hemolytic transfusion reactions?
Recipient antibodies to donor leukocytes leading to cytokine release
The antibody part is why it’s more common in pregnant women and those who’ve received blood before.
Using leukocyte-reduced blood can decrease the incidence of this.
What are the units of hemoglobin?
g/dL
What are the three types of hemophilia and their defective proteins?
A: VIII
B: IX
C: XI
What are the two types of HIT?
Type I: mild drop in platelets (~100) that occurs 1-2 days after receiving heparin, no increase in thrombosis
Type II: more significant drop in platelets (~50) that occurs 10 days after receiving heparin and causes thrombosis
In which types o sickle cell patients does splenic sequestration occur?
- Young children (<3 yo) who are homozygous (SS)
- Adults who are heterozygous (SC disease)
How long does it take to thaw FFP?
15-20 minutes
What is the dosing-to-percent repletion for hemophilia?
A: 1 U/kg raises 2% (812 VIII, 1 U, 2%)
B: 1 U/kg raises 1% (911 IX, 1 U, 1%)
In those with hemophilia who have a head injury with no bleeding on CT, what are the indications for treatment with supplemental factor?
All non-trivial head injuries should be treated with supplemental factor because of the risk of delayed bleeding. The goal is 100% factor for CHI and 40% factor for hemarthrosis and other bleeds.
Where is the most common site of Cushing tumors?
Pituitary followed by adrenal cortex
What does a T&S “screen” for?
Antibodies
What is the risk of getting HCV from a single unit blood transfusion?
1 in 3.1 million
Review the management of supratherapeutic INR in asymptomatic patients?
3.5 - 10: hold doses of warfarin and recheck
> 10: give 1-2.5 mg PO vitamin K and recheck in a few days
What is the management of elevated INR (from warfarin) in the setting of life-threatening bleed?
PCC and 10 mg IV vitamin K
How does TRALI differentiate from TACO in terms of acuity of onset?
TACO is usually more slow onset, whereas TRALI typically happens abruptly.
True or false: isolated fever during a blood transfusion requires no workup.
False
Isolated fever can be a finding of hemolytic transfusion reaction (from ABO incompatibility), especially if the fever is immediate. If they have an immediate fever then you need to check for ABO incompatibility and direct coombs.
Symptomatic hemolytic transfusion reactions include HA, nausea, hypotension, and hematuria.
How long does IV vitamin K take to normalize the INR?
4-6 hours
What are the advantages of PCC over FFP?
Smaller volume (better for CHF patients)
More rapid reversal (lower volume makes it easier to bolus)
True or false: large, nontender cervical lymphadenopathy for less than 1 week warrants biopsy in a child.
False
Cervical LAD is almost always associated with illness, especially in a child – even when non-tender. Must be present for weeks to warrant testing.
What paraneoplastic syndrome is associated with neuroblastoma?
Opsoclonus-myoclonus
How fast does hemoglobin usually increase after starting iron supplementation?
2-3 weeks
Dose of IX for heme B with suspected ICH?
100 IU/kg
Dose of VIII for heme A with suspected ICH?
50 IU/kg
Which sign is associated with pancreatitis, Trousseau or Lesser-Trelat?
Trousseau – migratory thrombophlebitis
“TRousseau TReks across the limbs.”
When does the nadir typically occur after chemotherapy?
6 - 10 days
Usually also recovers in 5 days
What are the leading causes of solid-organ cancer death and non-solid-organ cancer death?
Solid: lung adenocarcinoma
Non-solid: non-Hodgkins lymphoma
True or false: there is no platelet number in ITP in which platelet transfusion is indicated regardless of bleeding status.
False
Platelets less than 10k is an indication for transfusion regardless of bleeding status.
What therapy should you give in addition to recombinant factor for significant trauma or bleeding to those with hemophilia A or B?
Cryoprecipitate
