Heme/Onc

Question 1

Acute GVHD (within 100 days post BMT) Clincal presentation

Answer 1

-Skin: painful maculopapular rash that can progress to toxic epidermal necrolysis,
-GI: abdominal pain, diarrhea, and elevated liver enzymes (eg, bilirubin, alkaline phosphatase).

This syndrome typically occurs within a median time of 19 days after HCT.

Question 2

Acute GVHD: Risk factors

Answer 2

-human leukocyte antigen mismatch or unrelated donor
-gender disparity (eg, female donor to male recipient)
-lack of prophylactic GVHD regimen (eg, methotrexate, cyclosporine).

Question 3

Acute GVHD treatment

Answer 3

Steroids with cyclosporine and Methotrexate

Question 4

Chronic GVHD onset

Answer 4

Chronic GVHD resembles an autoimmune vascular disease and can occur in up to 50% of HCT patients at any time after transplant (median of 201 days post HCT).

Question 5

Chronic GVHD Risk factors

Answer 5

older age of donor/recipient,
prior acute GVHD,
seropositive cytomegalovirus in either donor or recipient.

Question 6

Chronic GVHD presentation

Answer 6

Skin: skin rash resembling lichen planus or scleroderma with areas of hypo- or hyperpigmentation.
GI: increased liver function tests (eg, bilirubin, alkaline phosphatase), dry oral mucosa with ulcerations, esophageal webs/strictures
Opth: dry eyes with possible cataracts
lung: bronchiolitis obliterans,
MSK: fasciitis or joint strictures.

Question 7

Chronic GVHD diagnosis

Answer 7

Diagnosis is usually clinical but may require skin or gastrointestinal biopsy

Question 8

Chronic GVHD treatment

Answer 8

Localized therapy (eg, topical steroids for skin involvement, cyclosporine eye drops) is preferred for limited chronic GVHD.

Extensive chronic GVHD requires systemic steroids with cyclosporine or tacrolimus.

Question 9

Haiy cell leukemia

Answer 9

Rare, mature B-cell lymphoproliferative disorder that typically causes splenomegaly and pancytopenia.

peripheral blood smear shows tumor cells that are 1-2 times the size of normal lymphocytes with “hairy” projections.

Confirmation is made with peripheral blood flow cytometry and trephine bone marrow biopsy with immunohistochemical stains.

Question 10

Type 1 heparin-induced thrombocytopenia

Answer 10

Non-immune-mediated thrombocytopenia due to an immediate (within 48 hours) reaction to heparin therapy.
Platelet counts rarely drop to <100,000/mm³ and usually recover spontaneously regardless of heparin continuation or discontinuation

Question 11

Acute intermittent porphyria

Answer 11

Patients presenting with recurrent acute abdominal pain combined with dark urine and hyponatremia should be evaluated for acute intermittent porphyria. The diagnostic test is an elevated urinary porphobilinogen (PBG) during an acute attack. Treatment involves glucose loading and intravenous hemin

Question 12

Tamoxifen preOp

Answer 12

It exerts estrogen- mimetic effects on the coagulation system and is associated with increased rates of venous thromboembolism (VTE).
Risk of VTE is particularly high during the first 2 years of therapy and in those who require prolonged immobilization due to surgery (or travel).
As a result, patients who take tamoxifen for primary prophylaxis should discontinue the medication 2-4 weeks prior to any surgery associated with a moderate or high risk of VTE (eg, hip replacement).
Those on tamoxifen for cancer treatment should discuss perioperative cessation with their oncologist.
Procedures associated with a low risk of VTE usually do not require tamoxifen cessation

Question 13

TLS management

Answer 13

Patients at high risk for tumor lysis syndrome from chemotherapy should be treated with aggressive hydration (to maintain a urine output of >80-100 mL/hr), rasburicase, and careful clinical and biochemical monitoring.

Question 14

CLL definition

Answer 14

Accounts for nearly 30% of adult leukemias in the United States and has a median age of 70 at diagnosis.

Patients typically present with “B symptoms” (eg, recurrent fever, night sweats, weight loss), painless lymphadenopathy, hepatosplenomegaly, anemia, and thrombocytopenia.

Diagnosis is confirmed by peripheral smear showing ≥5000/mm³ mature-appearing small B lymphocytes and flow cytometry showing monoclonal B-cell characteristics (eg, CD19, CD20, CD23).

Question 15

CLL indication of treatment

Answer 15

CLL is usually an indolent disease with a median survival of >10 years in asymptomatic patients with early-stage disease (Rai stage 0-1, Binet stage A). As a result, asymptomatic patients do not need treatment and can be observed for possible disease progression

Indication:
• Systemic symptoms (eg, fever, weight loss, fatigue, night sweats)
• Massive/progressive lymphadenopathy or hepatosplenomegaly
• Progressive marrow failure (eg, anemia, thrombocytopenia)
• Autoimmune hemolytic anemia or thrombocytopenia unresponsive to steroids
• Rapidly progressive lymphocytosis (doubling time <6 months)
• Repeated infections

Question 16

Post Radiation screening

Answer 16

In patients who received chest radiation, annual screening mammograms are recommended starting 8-10 years after radiation therapy. Annual TSH testing is also recommended in these patients.

Question 17

Complex regional pain syndrome

Answer 17

Complex regional pain syndrome commonly presents after an injury with throbbing pain, paresthesias, skin temperature changes, and local edema. Diagnosis is suggested clinically by symptoms and abrupt symptom relief with a local anesthetic block.

Question 18

Dabigatran pre-op

Answer 18

The ongoing physiologic effects of dabigatran, a direct thrombin inhibitor used as an oral anticoagulant, can be assessed using thrombin time:

A)Normal thrombin time: The patient is not anticoagulated surgery can proceed without additional interventions.

B)Elevated thrombin time: The patient is currently anticoagulated administration of the dabigatran antidote idarucizumab should be considered in patients with life- threatening bleeding and those who require urgent/emergency surgery.

Patients on dabigatran undergoing elective procedures are generally advised to discontinue the medication 48 hours prior to the operation (as the half-life is approximately 12 hours). These individuals usually do not require a thrombin time

Question 19

Post polycythemia Vera myelofibrosis
Diagnostic criteria

Answer 19

Major
• Previous diagnosis of PV
• Presence of increased bone marrow fibrosis
Minor
• Progressive anemia, loss of requirement for phlebotomy, or cytoreductive therapy
• Leukoerythroblastic blood picture
• Increasing splenomegaly
• Development of constitutional symptoms (fever, weight loss, and night sweats)

Treatment for post-PV myelofibrosis is similar to that for primary myelofibrosis. The only definitive treatment is allogenic hematopoietic stem-cell transplantation. Patients with severe splenomegaly and debilitating constitutional symptoms can be treated with JAK2 inhibitors (eg, ruxolitinib).

Question 20

Priapism in sickle cell management

Answer 20

Priapism is a serious complication associated with sickle cell disease and defined as an erection lasting >2-4 hours. Blood aspiration from the corpus cavernosum, followed by saline irrigation and use of adrenergic agonists (eg, epinephrine), is first-line therapy. If priapism still persists, surgical shunting may be required.

Question 21

Transfusion associated GVHD
Introduction

Answer 21

a rare condition that occurs when donor lymphocytes from transfused blood attack host tissue. Nearly all cases arise in patients who are immunocompromised

Unlike GVHD associated with hematopoietic stem cell transplantation, ta-GVHD causes bone marrow destruction leading to progressive pancytopenia, which is the major underlying cause of morbidity and death.

Question 22

Transfusion associated GVHD
Presentation and treatment

Answer 22

Manifestations typically occur 4-30 days after transfusion and include fever and an erythematous/maculopapular rash that often becomes generalized; additional features include anorexia, (right upper-quadrant) abdominal pain, hepatomegaly, liver function abnormalities, and profuse water diarrhea (>7-8 L/day).

Treatment is typically ineffective, and most cases (80%-90%) are fatal. Therefore, prevention is paramount. Those at high risk for the condition (eg, immunocompromised state) should receive irradiated blood products to inactivate donor lymphocytes prior to transfusion. Patients with HIV do not appear to be at risk for this condition and can receive nonirradiated blood (possibly because HIV alters donor lymphocyte survival)

Question 23

Borderline Vit B12

Answer 23

Vitamin B12 deficiency is common in patients with advanced age and may present with neuropsychiatric findings and no hematologic findings. Laboratory evaluation that reveals a borderline vitamin B12 level requires further testing with methylmalonic acid (MMA) level. A high level of MMA confirms vitamin B1, deficiency.

Question 24

Multiple myeloma with negative SPEP

Answer 24

Multiple myeloma (MM) is usually associated with a monoclonal spike on serum protein electrophoresis (SPEP). However, this test often is unable to identify patients who produce excessive light chains rather than full immunoglobulin. Therefore, patients with classic symptoms of MM who have negative SPEP should undergo urine electrophoresis with immunofixation or serum free light-chain assay, which can identify monoclonal light chains.

Patients with negative SPEP, UPEP, and serum FLC analysis who have classic symptoms of MM and bone marrow biopsy evidence of ≥10% clonal plasma cells have “nonsecretory” myeloma, which is seen in ~3% of cases.

Question 25

Sickle cell and strokes

Answer 25

Patients with sickle cell disease are at high risk for ischemic or hemorrhagic stroke. Exchange transfusion is recommended for acute stroke; scheduled simple transfusions are recommended to prevent recurrent strokes. Done every 4-6 weeks with goal Hb S levels of <30% and total Hb levels of 9-12.5 mg/dL. This strategy reduces the risk of stroke recurrence from 65% to <20% and is superior to medical management with hydroxyurea.

Question 26

Post orthopedic surgery VTE prophylaxis

Answer 26

For patients undergoing orthopedic surgery without increased bleeding risk, postoperative dual venous thromboembolism prophylaxis with intermittent pneumatic compression and low-molecular-weight heparin is recommended during hospitalization; low-molecular-weight heparin should be continued for up to 35 days.

Question 27

Myeloid reconstitution syndrome

Answer 27

Previously febrile neutropenic patients experiencing myeloid recovery may develop recurrent fever and the appearance or worsening of an inflammatory focus, a condition known as myeloid reconstitution syndrome. Although affected patients should be closely monitored for infection, no immediate treatment change is typically required.

Question 28

Polycythemia

Answer 28

Polycythemia is defined as a hemoglobin level >16 g/dL in women and >16.5 g/dL in men and is typically categorized into primary and secondary causes. Primary polycythemia is erythropoietin (EPO)-independent and is usually associated with low EPO levels; polycythemia vera is the most common cause and is usually diagnosed by JAK2 mutation testing and bone marrow biopsy. Secondary polycythemia is usually EPO-dependent and marked by high EPO levels; hypoxia is the most common cause and is typically diagnosed by oxygen saturation testing at rest, after exercise, and during sleep.

Question 29

Renal cell carcinoma Signs and symptoms

Answer 29

• Can be asymptomatic • Hematuria (only with invasion of collecting system) • Abdominal mass, abdominal pain, weight loss • Scrotal varicocele

Question 30

Renal cell carcinoma Para neoplastic syndrome

Answer 30

• Anemia (microcytic or normocytic) • Erythrocytosis (increased erythropoietin production) • Nonmetastatic liver function abnormalities • Fever & weight loss • Hypercalcemia (due to bone metastases,↑ PTHrP, or ↑ prostaglandins) • AA amyloidosis

Question 31

Erythromelalgia Definition and presentation

Answer 31

a condition that causes burning pain and erythema (sometimes pallor and/or cyanosis) in the extremities. It is likely due to intermittent blood vessel obstruction. Episodes are usually triggered by heat, exertion, stress, insomnia, or pressure. Symptoms can occur in the symmetric extremities (lower > upper), ears, or face. Patients typically avoid wearing socks or shoes since the generated heat can precipitate attacks. Elevating the legs can also help relieve symptoms

Question 32

Erythromelalgia Causes and treatment

Answer 32

Erythromelalgia can be either primary or secondary, and is usually due to an underlying myeloproliferative disorder with platelet counts > 400,000 cells/µl. Diagnosis is usually clinical as there are no specific diagnostic tests. Treatment is directed at the underlying disorder (if secondary), but aspirin may reduce symptoms in both primary and secondary erythromelalgia. Aspirin is also helpful in reducing vascular events in patients with essential thrombocythemia. In addition, patients are instructed to elevate their extremities, cool the affected area, and avoid heat or any other triggers.

Question 33

Prostate cancer radiation complications

Answer 33

Sexual dysfunction is the most common long-term adverse effect of external beam radiation for prostate cancer. The risk depends on the radiation technique and radiation field. The risk increases further if androgen deprivation therapy is added to the radiation treatment.

Question 34

Small cell lung carcinoma management

Answer 34

Patients with SCLC are categorized based on radiograph and physical examination findings as follows: Limited stage - tumor limited to the ipsilateral hemithorax and regional lymph nodes (although subclinical metastases are usually present) Extensive stage - involvement of contralateral lymph nodes, malignant pericardial/pleural effusion, or distant metastases (as in this patient with liver and contralateral lung lesions) Platinum-based chemotherapy (eg, etoposide plus cisplatin) is highly effective for inducing remission in SCLC. Patients with limited-stage SCLC are treated with curative intent and usually receive concurrent chemotherapy and thoracic radiation; 5-year survival is ~20%. Extensive-stage SCLC is generally treated with 6 cycles of chemotherapy alone; those who respond to chemotherapy often receive subsequent chest and/or prophylactic cranial irradiation (Choice E). Five-year survival is ~5%. targeted drug therapy is not currently part of first-line treatment for SCLC, molecular profiling is not typically performed.

Question 35

Delayed onset HIT

Answer 35

Delayed-onset, heparin-induced thrombocytopenia is an immune-mediated condition that usually arises 5-19 days after heparin cessation. Most patients present in the outpatient setting with thrombocytopenia and arterial or venous thrombus. HIT antibody testing is required for confirmation, but empiric treatment with a nonheparin anticoagulation (eg, direct thrombin inhibitor) should be initiated with urgency to prevent life-threatening complications.

Question 36

Breast cancer workup

Answer 36

Imaging studies for staging are not recommended in asymptomatic patients with newly diagnosed stage 0 to II breast cancer. Brain metastasis occurs in only a small proportion of patients with metastatic breast cancer and generally later in the disease course.

Question 37

Colon cancer management

Answer 37

• The FOLFOX regimen of 5-fluorouracil, leucovorin, and oxaliplatin or oxaliplatin with oral capecitabine is the most appropriate adjuvant therapy for stage III colon cancer. • Unlike rectal cancer, adjuvant irradiation is not part of the routine management of colon cancer.

Question 38

Lymphoma and LDH

Answer 38

Patients with higher LDH levels have a poorer prognosis, and the result is used in calculating the patient's International Prognostic Index score, which incorporates LDH along with age, stage, performance status, and the presence or absence of extra- nodal involvement. The International Prognostic Index score correlates with progression free and overall survival after standard therapy. Patients with poor prognosis may be considered for more aggressive treatment.

Question 39

Cervical cancer management

Answer 39

Hysterectomy and pelvic node dissection are recommended for patients with stage I and II cervical cancer but not for patients with stage III disease. Patients with bulky or locally advanced stage III cervical cancer are treated with cisplatin-based chemo- therapy and radiation therapy.

Question 40

CLL and recurrent infection

Answer 40

• In patients with chronic lymphocytic leukemia, repeated infections, and hypogammaglobulinemia, regular treatment with intravenous gamma globulin reduces infectious events. Seasonal influenza vaccinations as well as 13-valent conjugate vaccine and 23-valent polysaccharide vaccine should be given to patients with chronic lymphocytic leukemia.

Question 41

Work up for re-occuring breast cancer

Answer 41

• Breast cancer can undergo subtype switch between the primary disease and metastatic disease, and biopsying the metastatic site allows treatment to be tailored to the metastatic disease subtype. • When evaluating a patient with newly diagnosed metastatic breast cancer, the lesion that upstages the patient to the greatest degree should be biopsied.

Question 42

Non small cell lung cancer management

Answer 42

• In the absence of a driver mutation (epidermal growth factor receptor, ALK, ROS1), combination of pembrolizumab with chemotherapy has been shown to improve survival in patients who have good performance with metastatic non-small cell lung cancer regardless of programmed cell death ligand-1 level. • In the absence of a driver mutation (epidermal growth factor receptor, ALK, ROSI), pembrolizumab monotherapy is first-line therapy in patients with programmed cell death ligand-1 expression greater than 50%

Question 43

Low grade lymphoma transformation

Answer 43

Histologic transformation of follicular lymphoma is suggested by new onset of systemic symptoms, declining performance status, lactate dehydrogenase or calcium elevation, new sites of extranodal disease, or characteristic CT/PET findings. Suspected histological transformation of low-grade non-Hodgkin lymphoma should be confirmed by lymph node biopsy. Transformation has important prognostic and therapeutic implications, as such events indicate a worse prognosis and require more aggressive therapy. If his lymphoma has undergone transformation to a higher grade of disease, combination anthracycline-based chemotherapy, autologous hematopoietic stem cell transplantation, and in some cases, CD19-targerted CAR-T cell therapy would be a treatment option.

Question 44

Differentiation syndrome

Answer 44

life-threatening complication seen in patients with APL treated with ATRA or arsenic trioxide. It is characterized by fever, fluid retention, dyspnea, pleural effusions, pulmonary infiltrates, and organ dysfunction. The condition is treated with high-dose corticosteroids (e.g., dexamethasone). Early recognition and prompt initiation of corticosteroids are crucial to prevent severe complications.

Question 45

Management of polycythemia Vera

Answer 45

🔴Phlebotomy to hematocrit < 45% and Aspirin in all pt. 🔴Cytoreduction with hydroxyurea in high risk disease: age > 60y or hx of thrombosis.

Question 46

CLL treatment indications

Answer 46

🔴 constitutional symptoms 🔴 Progressive lymphadenopathy 🔴 Hepatosplenomegaly 🔴 Increases in lymphocyte count > 50% in 2 months or doubling on less than 6 months 🔴 Anemia hb<10 🔴 Thrombocytopenia Plt <100 🔴 Bone marrow failure 🔴 Extra nodal involvement

Question 47

TKI toxicity

Answer 47

🔴Imatinib, Renal and neurotoxicity 🔴Dasatinib, pulmonary hypertension, and pleural effusion 🔴Nilotinib, VTE renal toxicity and pancreatitis 🔴Ponatinib, VTE, arterial thrombosis and refractory hypertension

Question 48

When to use Ponatinib

Answer 48

The T3151 mutation confers resistance to most first- and second- generation TKIs (like imatinib, dasatinib, and nilotinib). Ponatinib is a third-generation TKI specifically designed to target the T3151 mutation, making it the most appropriate treatment for patients with this mutation

Question 49

Predictor of good response to ESA in patients with MDS

Answer 49

Serum EPO < 500 U/I and transfusion needs of < 2 units/month predict a high probability of response to ESA therapy.

Question 50

MDS management

Answer 50

Low risk 🔴Anemia →EPO+/-GCSF. 🔴5q del →Lenalidomide. 🔴SF3B1 →Luspatercept 🔴Hypoplastic→ ATG/Cyclosporine (Transfusion with iron chelating) High risk 🔴transplant candidate → BMT. 🔴non transplant → Hypomethylating agent (azacitidine or decitabine)

Question 51

Myeloma kidney

Answer 51

a common cause of renal impairment in patients with multiple myeloma. It occurs when free light chains are filtered by the kidneys and precipitate in the renal tubules. These light chains form obstructive casts that block the tubules, leading to inflammation, tubular injury, and eventually, kidney damage.

Question 52

Poor Prognostic Factors in Acute Lymphoblastic Leukemia (ALL):

Answer 52

🔴Philadelphia chromosome (t[9;22], BCR-ABL1) 🔴MLL rearrangement (KMT2A gene) 🔴Hypodiploidy 🔴Minimal Residual Disease (MRD) positivity

Question 53

**Wild-Type Transthyretin Amyloidosis (ATTRwt):**

Answer 53

- ATTRwt involves the deposition of normal transthyretin (TTR) protein, mainly affecting older adults. - It primarily leads to restrictive cardiomyopathy (heart failure with preserved ejection fraction), often with associated carpal tunnel syndrome and other musculoskeletal issues. - Diagnosis typically involves cardiac imaging and biopsy or cardiac scintigraphy. - Treatment includes supportive care and TTR stabilizers like *tafamidis* (might come in exam)

Question 54

**Systemic Amyloidosis:**

Answer 54

- A group of disorders characterized by the deposition of misfolded protein fibrils in various organs. - Major types include AL (light chain), AA (inflammatory-related), hereditary (mutant TTR), and dialysis-related amyloidosis. - Symptoms depend on the organs involved, such as the heart, kidneys, liver, and nervous system. - Diagnosis involves tissue biopsy and imaging, while treatment focuses on managing the underlying cause and organ-specific care.

Question 55

Approved indication for CAR-T therapy Target antigen CD19

Answer 55

🔴B ALL 🔴B NHL

Question 56

Follicular lymphoma treatment

Answer 56

First line of treatment is watch and wait Indications of treatment as follows: 🔴 B symptoms (unexplained fever >38 C, drenching night sweats or loss of >10% body weight within 6 months) 🔴 hematopoietic impairment, 🔴 bulky disease, vital organ compression, or rapid lymphoma progression

Question 57

Lymphoma chemotherapy

Answer 57

🔴 Hodgkin's lymphoma ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine 🔴 Non Hodgkin lymphoma (most common type is DLBCL) R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)

Question 58

Acute intermittent porphyria (AIP)

Answer 58

🔴triad of abdominal pain, CNS abnormalities and peripheral neuropathy 🔴caused by porphobilinogen deaminase deficiency. 🔴Autosomal dominant disease with (low penetrance). 🔴Always look for exacerbating factors: medications (almost always antiseizure), menstrual cycle, alcohol, fasting and stress. 🔴Diagnosis: urine porphobilinogen.

Question 59

Treatment of AIP

Answer 59

Severe: Hemin (can be given to pregnant patients) Mild: IV hemin Vs carbohydrate loading (IV dextrose) Prophylaxis: 🔴More than 4 attackes per year: Givosiran. 🔴GnRH agonists for menstrual induced attacks. Definitive treatment: liver transplant

Question 60

AIP and HCC

Answer 60

patients with AIP have an increased risk for hepatic carcinoma after the age of 50, and they should have surveillance tests performed regularly. Surveillance tests include performing a liver ultrasound and alpha- fetoprotein every 6-12 months

Question 61

Thalassemia

Answer 61

Alpha trait Microcytic anemia + normal hemoglobin electrophoresis Beta thalassemia Has elevated HbA2 in electrophoresis>4%

Question 62

Pregnancy and sickle cell disease

Answer 62

🔴Vaso-occlusive crises are more common in pregnancy. 🔴SCD is NOT a contraindications to vaginal delivery. 🔴Hormonal contraceptives and copper-releasing intrauterine device are safe and effective methods for women with SCD 🔴 Morphine is analgesic of choice during pregnancy

Question 63

TTP in a nutshell

Answer 63

Scenario: Inciting factor (pregnancy, clopidogrel, surgery) followed by drop in platelet count and schistocytes in peripheral blood morphology. To confirm the diagnosis you need to measure ADAMTS13 level. Treatment: plasma exchange +/- steroid & Rituximab. > Caplacizumab which is an anti-VWF has been recently approved for treatment of TTP.

Question 64

Hemochromatosis and infections

Answer 64

Iron overload of macrophages can cause impaired phagocytosis and lead to decreased immunity, resulting in an increased risk of infection from Listeria, Yersinia enterocolitica, and Vibrio vulnificus

Question 65

Hemochromatosis complications reversibility

Answer 65

🔴Cardiomyopathy and skin pigmentation are reversible complications. 🔴Diabetes mellitus, arthropathy, liver cirrhosis, and hypogonadotropic hypogonadism are irreversible complications of hemochromatosis

Question 66

Management of hemophilia bleed

Answer 66

Mild⏩ desmopressin Mod/severe ⏩ Factor VIII replacement

Question 67

ITP treatment

Answer 67

One should not treat a patient with ITP unless there are signs of active bleeding or if there is a very low platelet count <30,000 1st line Steroids +/-IVIG If no response; splenectomy, rituximab, or TPO agonists

Question 68

Argatroban

Answer 68

🔴Argatroban is a direct thrombin inhibitor 🔴therapy is monitored using the aPTT with a target range of 1.5 to 3 times the initial baseline value (not to exceed 100 seconds). 🔴Argatroban fa'sely elevates INR, due to an interaction with the thromboplastin reagent. 🔴Prothrombin time (PT) and thrombin time (TT) are affected by argatroban as well; however, the therapeutic ranges for these tests have not been identified

Question 69

IVC filter insertion indication

Answer 69

🔴Acute VTE with contraindication to anticoagulation (e.g. active bleeding). 🔴Patients who develop a pulmonary embolism despite therapeutic anticoagulation, 🔴Patients with a massive PE and poor cardiopulmonary reserve. If a filter is needed, then a retrievable filter should be placed. The filter should be removed when the risk for PE has resolved and/or when anticoagulation can be safely resumed.

Question 70

Protein c deficiency and warfarin

Answer 70

heterozygous or homozygous protein C deficiency. Individuals with hereditary protein C deficiency lack the natural anticoagulant function of activated protein C and are at risk for clinical phenotypes associated with increased thrombotic risk including VTE, warfarin- induced skin necrosis, and (in patients with homozygote disease) neonatal purpura fulminans

Question 71

Management of warfarin induced skin necrosis

Answer 71

For a patient who develops warfarin-induced skin necrosis, immediate intervention is required to prevent rapid progression of the necrosis and to minimize complications. 🔴 Warfarin must be stopped immediately. 🔴 Vitamin K and therapeutic heparin anticoagulation is provided for anticoagulation. 🔴 Lastly, Protein C concentrate or FFP should be given.

Question 72

Anticoagulation for HIT

Answer 72

--Parenteral direct thrombin inhibitors (argatroban, bivalirudin) --Fondaparinux: Anti Xa inhibitor. --Direct oral anticoagulants (apixaban, edoxaban, rivaroxaban, dabigatran

Question 73

When to start warfarin in patients with HIT

Answer 73

If the decision is to shift the patient to warfarin, warfarin should be started AFTER platelet recovery (platelet >150,000

Question 74

Duration of anticoagulation in HIT

Answer 74

> No thrombosis: 4 weeks. > Thrombosis 3 months

Question 75

Leuco-irradiation

Answer 75

Irradiation is aimed at reducing the risk of transfusion-related graft-versus-host disease. It is a disease associated with transfusion of donor T cells which proliferate and attack the recipient. Indications: -Individuals with congenital immunodeficiency states -Individuals treated with intensive chemotherapy. -Recipients of hematopoietic stem cell transplant (autologous or allogeneic) -Individuals at risk for partial HLA matching with the donor due to directed donations, HLA-matched products, or genetically homogeneous populations.

Question 76

Bacterial associated transfusion reaction

Answer 76

The signs of a bacterial associated transfusion reaction include an elevated fever, hypotension, and tachycardia. Platelets are associated with the greatest risk of transfusion- associated bacterial infection due to their storage. At room temperature (only viable for 5 days)

Question 77

Washed RBC

Answer 77

Anaphylactic reaction following transfusion of blood products can occur in patients with IgA deficiency. Washing the red blood cell reduces the immunoglobulins including IgA. This reduces the risk of allergic/anaphylactic reaction in these patients when they are transfused blood.

Question 78

Dilution coagulopathy

Answer 78

Dilutional coagulopathy refers to alterations in the coagulation system that are induced by a patient receiving large volumes of intravenous fluids or unbalanced component blood administration. Massive transfusion occurs when there is replacement of a patient's blood volume in <24 hours, it takes about 10 pRBC transfused in <24 hours to cause this. It can lead to coagulopathy, hypocalcemia and hypothermia. This patient should have a DIC panel sent for. If dilutional coagulopathy is confirmed, then the patient should receive FFP infusions

Question 79

Fresh frozen plasma pearls

Answer 79

🔴It has the highest association with TRALI 🔴Indication A) TTP/HUS (if plasmapheresis is not available). B) Massive transfusions C) PT/PTT 1.5 ULN with active bleeding. D) Reversal of warfarin overdose in bleeding patient (PCC is superior). 🔴Goals of FFP administration: 1 Keep the fibrinogen > 100 mg/dL 2. PT/PTT should be < 1.5 the normal value

Question 80

Leuco-reduction

Answer 80

Leukoreduction can take place on both red blood cell and platelet product. This removes the vast majority of white cells in the blood product. Indications --Reduce HLA alloimmunization to leukocyte antigens. This includes transplants candidates and those patients who are transfusion-dependent --Reduce the rate of CMV transmission --Reduce febrile non-hemolytic transfusion reactions

Question 81

Pure red aplasia

Answer 81

• Pure red cell aplasia is characterized by normocytic or macrocytic anemia with decreased reticulocytes and absent or decreased erythrocyte precursors in the bone marrow. • Immunocompromised patients can have sustained parvovirus viremia, leading to acquired pure red cell aplasia and prolonged anemia requiring intravenous immune globulin to hasten viral clearance.

Question 82

Herpes and multiple myeloma

Answer 82

Treatment guidelines for patients with multiple mye- loma recommend that those treated with proteasome inhibitors (e.g., bortezomib) should receive antiviral prophylaxis (acyclovir, valacyclovir) to avoid herpes zoster virus reactivation.

Question 83

Thrombocytopenia in pregnancy Gestational vs ITP

Answer 83

Features suggesting immune thrombocytopenic purpura in pregnancy include, earlier presentation (first trimester), lower platelet count nadir (< 70 / mu * L [70 * 10 deg / L] ). and history of thrombocytopenia before pregnancy. Gestational thrombocytopenia presents late in ges- tation, with platelet counts typically greater than 100000 / mu * L (100 * 10 ^ 9 / L) although platelet counts may reach a nadir of 70,000/µl. (70 * 10 ^ 9 / L) plate let counts spontaneously return to normal after delivery

Question 84

Evaluation of splanchnic vein thrombosis

Answer 84

• In patients with splanchnic vein thrombosis (portal vein, splenic vein, hepatic vein, or mesenteric vein thrombosis), evaluation for evidence of a myeloprolif erative neoplasm should be considered, including evaluation for the JAK2 tyrosine kinase mutation. • Evidence of a myeloproliferative neoplasm is discov- ered in approximately 50% of patients with Budd- Chiari syndrome, even when the complete blood count is normal.

Question 85

Aplastic anemia management

Answer 85

• Allogeneic hematopoietic stem cell transplantation is the preferred treatment for aplastic anemia in younger patients who have an HLA-matched stem cell donor. • In patients older than 50 years and in younger patients without a suitable stem cell donor, aplastic anemia is treated by immunosuppression with antithymocyte globulin, cyclosporine, and prednisone.

Question 86

Essential thrombocythemia management

Answer 86

Aspirin for everybody. (To suspend if there are signs of aquired vWD) Indication for cytoreduction: -Age >60 -History of thrombosis Agent of choice is hydroxyurea. Intrrferon alpha if pregnant

Question 87

Cold hemolytic anemia

Answer 87

🔴In patients with hemolytic anemia and a positive direct antiglobulin test to anticomplement (C3) but not IgG, the diagnosis of cold agglutinin disease can by confirmed by a cold agglutinin titer. 🔴Cold agglutinin hemolytic anemia can arise as a primary process or secondary to an underlying condition such as infection (mycoplasma), lymphoproliferative disorders, or autoimmune disease.

Question 88

Superficial venous thrombosis management

Answer 88

🔴Six weeks of anticoagulation is indicated for superfi- cial vein thrombosis when the thrombus is 5 cm or greater in length or is close to the deep venous system or when other thrombophilic risk factors present. 🔴For patients with low-risk superficial vein thrombo- sis, observation with follow-up in 1 week to ensure symptom resolution is reasonable; if symptoms persist or worsen, repeat duplex ultrasonography should be performed. If repeat ultrasound shows thrombus extension, anticoagulation would then be indicated.

Question 89

Coagulopathy of liver disease vs DIC

Answer 89

• Factor VIII is produced in hepatic and nonhepatic endothelial cells; it is normal or elevated in the coagulopathy of liver disease and is consumed in disseminated intravascular coagulation.