heme/onc 6 Flashcards
What is melanoma?
Melanoma is a type of skin cancer that develops from the pigment-producing cells known as melanocytes.
What is renal-cell carcinoma?
Renal-cell carcinoma is a type of kidney cancer that originates in the lining of the renal tubules.
What types of cancer are associated with BCR-ABL rearrangement?
BCR-ABL rearrangement is common in chronic myeloid leukemia (CML).
What is a serum tryptase level indicative of?
A serum tryptase level >20 ng/mL in the absence of an associated myeloid disorder is a minor diagnostic criterion for systemic mastocytosis.
What factors are thought to precipitate porphyria cutanea tarda (PCT)?
Multiple risk factors contribute to PCT by causing iron accumulation and/or oxidative stress in hepatocytes.
What is hereditary hemochromatosis (HH)?
HH is most commonly caused by mutations in the HFE gene, such as C282Y or H63D, and typically manifests in middle-aged white men.
What is secondary hemostasis?
Secondary hemostasis is the stabilization of the platelet plug (white thrombus) by creating a fibrin network.
What is the most likely diagnosis for a young adult with a destructive bony lesion near the knee?
The most likely diagnosis is osteosarcoma, especially with radiographic findings consistent with a sunburst pattern.
What is the best initial management for patients with metastatic cancer and intractable pain?
The best initial management is a patient-controlled analgesia pump with an intravenous opioid.
What drug has been associated with significant improvements in overall survival among patients receiving carboplatin and paclitaxel?
Bevacizumab has been associated with significant improvements in overall survival among these patients.
What are myeloproliferative neoplasms (MPNs)?
MPNs are the most common acquired risk factor for Budd-Chiari syndrome.
What effect do diminished levels of von Willebrand factor have?
Diminished levels of von Willebrand factor increase clearance of factor VIII and lead to a factor VIII deficiency.
What virus is associated with hepatitis C?
Hepatitis C virus (HCV) can lead to various complications, including liver disease.
What virus is associated with the development of lymphomas?
HCV is associated with the development of lymphomas of B-cell origin, particularly splenic lymphoma.
What is the most common type of sarcoma in children and adolescents?
Ewing sarcoma is most common in children and adolescents.
What reagent should be used to assess for pseudothrombocytopenia?
To assess for pseudothrombocytopenia, repeat testing with citrate instead of EDTA.
What does EDTA cause in patients?
EDTA causes the development of platelet autoantibodies resulting in clumping.
What is the most appropriate management for cancer pain crisis with opioid-induced neurotoxicity?
The most appropriate management is opioid rotation with dose reduction to account for incomplete cross-tolerance.
What may beta-thalassemia intermedia require?
Beta-thalassemia intermedia may require intermittent blood transfusions.
Where are varicoceles fairly common?
Varicoceles are fairly common on the left side because of the higher pressure within the left gonadal vein compared with the right gonadal vein.
What does beta-thalassemia lead to?
Beta-thalassemia leads to abnormal erythrocytes that express procoagulant phospholipids, activated clotting factors, and activated platelets, all of which predispose patients to thrombosis.
What is the most appropriate treatment for diarrhea and flushing associated with carcinoid syndrome?
The most appropriate treatment is a somatostatin analogue such as octreotide.
What does hydroxyurea increase?
Hydroxyurea increases levels of hemoglobin F.
What is the most likely diagnosis in a patient with pancytopenia without myeloid immaturity?
The most likely diagnosis is Gaucher disease.
What is the likely diagnosis in an asymptomatic person with a mean corpuscular volume in the 60s but normal hemoglobin?
The likely diagnosis is beta-thalassemia trait or alpha-thalassemia trait.
What happens to von Willebrand factor levels in certain conditions?
Diminished levels of von Willebrand factor increase.
What factor is diminished in von Willebrand disease?
Diminished levels of von Willebrand factor.
What does increased clearance of factor VIII lead to?
A deficiency in factor VIII.
How does the left gonadal vein drain?
It drains at a right angle into the left renal vein.
How does the right gonadal vein insert?
It inserts into the inferior vena cava at a more acute angle.
What is the most appropriate management of hypercalcemia of malignancy?
Aggressive volume repletion followed by either an intravenous bisphosphonate or denosumab.
What are superwarfarins?
High-potency vitamin K antagonists used as rat poisons.
What is the most likely cause of night sweats, weight loss, and cervical lymphadenopathy in a patient with common variable immunodeficiency?
Non-Hodgkin lymphoma.
What features are associated with increased risk for progression of MGUS?
A serum monoclonal protein level >1.5 g/dL, non-immunoglobulin G monoclonal protein, and a markedly abnormal free-light-chain-kappa-to-lambda ratio.
What is TRALI commonly associated with?
Hypotension and normal/elevated central venous pressure.
What is the recommended follow-up for a smoker with a single solid pulmonary nodule 6 to 8 mm in size?
A follow-up low-dose CT scan of the chest in 6 months.
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
Intravenous immunoglobulin or stimulation of platelet production with eltrombopag or romiplostim, or immunosuppression with rituximab or other immunosuppressive agents.
What is reserved for patients refractory to medical management in ITP?
Splenectomy.
What forms a complex in the extrinsic pathway of coagulation?
Factor VIIa and tissue factor.
What is thrombocytosis often seen in?
Beta thalassemia patients due to increased drive on the marrow from erythropoietin.
What are dabigatran and rivaroxaban?
Anticoagulants.
Which anticoagulants are not recommended in patients with end-stage kidney disease?
Rivaroxaban and apixaban are not recommended in patients with end-stage kidney disease.
What is likely in patients with chronic kidney failure?
Functional iron deficiency is considered to be likely in patients with chronic kidney failure.
What ferritin level indicates functional iron deficiency in chronic kidney failure?
A ferritin level of ≤500 ng/mL indicates functional iron deficiency in chronic kidney failure.
What transferrin saturation level indicates functional iron deficiency?
A transferrin saturation of ≤30% indicates functional iron deficiency.
What is the difference in RDW between beta thalassemia and iron deficiency anemia?
In beta thalassemia, RDW is normal; in iron deficiency anemia, RDW is elevated.
When are erythropoiesis-stimulating agents started?
Erythropoiesis-stimulating agents are started when the hemoglobin level falls below 10 g/dL.
What hemoglobin level is critical to avoid falling below?
The hemoglobin concentration should not fall below 9 g/dL.
What are the most common primary tumors that metastasize to the brain?
The most common primary tumors that metastasize to the brain are lung cancer, melanoma, renal-cell carcinoma, and breast cancer.
What condition is indicated by plasmacytoid lymphocytes in the bone marrow?
Plasmacytoid lymphocytes in the bone marrow indicate Waldenström macroglobulinemia.
What does the treatment of TLS include?
The treatment of TLS includes rasburicase and aggressive volume expansion with normal saline.
What is the target urine output for most adults during TLS treatment?
The target urine output is around 150–200 mL/hour for most adults.
What is an important cause of chronic diarrhea following partial pancreatectomy?
Exocrine pancreatic insufficiency is an important cause of chronic diarrhea following partial pancreatectomy.
What is the best laboratory test to diagnose ACTH-associated paraneoplastic syndrome?
The best laboratory test is an overnight dexamethasone suppression test or a 24-hour urinary free cortisol test.
What is checked after administering dexamethasone in the overnight test?
The morning cortisol level is checked after administering 1 mg of dexamethasone at 11 p.m. the night before.
What indicates leukocytosis in a patient?
Leukocytosis is indicated by a white blood cell count above 100,000 cells per mm³ with absolute basophilia.
What is the most likely diagnosis in a patient with leukocytosis above 100,000 cells per mm3 with absolute basophilia, thrombocytosis, and splenomegaly?
The most likely diagnosis is chronic myeloid leukemia.
What is the classic appearance of meningiomas on MRI?
Meningiomas are adjacent to bone and anchored to the dura, rendering the classic appearance of the ‘dural tail’ on imaging.
What condition is characterized by immune-mediated necrotizing vasculitis affecting small and medium-sized vessels in the upper and lower respiratory tract, eyes, and kidneys?
Likely diagnosis: Granulomatosis with polyangiitis.
What is the diagnosis associated with a thrombotic event occurring in the context of persistently positive lupus anticoagulant or anticardiolipin antibody?
Diagnosis: Antiphospholipid syndrome.
What is the characteristic development timeline for therapy-related acute myeloid leukemia?
Classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
What is the most likely diagnosis in a young adult with a destructive bony lesion near the knee and radiographic findings consistent with a sunburst pattern?
The most likely diagnosis is osteosarcoma.
What is the risk category for patients with acute lymphocytic leukemia and a leukocyte count >50,000 cells per mm3?
They are in the highest risk category for tumor lysis syndrome.
What is Bevacizumab?
Bevacizumab is a monoclonal antibody against vascular endothelial growth factor.
What is the characteristic erythrocyte morphology in patients with beta-thalassemia?
Patients have uniformly microcytic erythrocytes.
What is the most likely diagnosis in a patient with splenomegaly and teardrop-shaped erythrocytes, nucleated erythrocytes, and immature granulocytes?
The most likely diagnosis is primary myelofibrosis.
What is primary myelofibrosis?
Primary myelofibrosis is a type of blood cancer that affects the bone marrow.
What is acute promyelocytic leukemia sensitive to?
Acute promyelocytic leukemia is very sensitive to vitamin A.
What are the derivatives that contribute to excellent long-term survival in acute promyelocytic leukemia?
The derivatives are all-trans retinoic acid and arsenic.
From where can squamous-cell carcinomas originate?
Squamous-cell carcinomas can originate from the skin, the mucosa of the upper aerodigestive tract, and the cervix.
What are examples of locations where squamous-cell carcinomas can occur?
Examples include the head, neck, and esophagus.
What is the most appropriate dose of short-acting opioids for breakthrough pain in patients with uncontrolled cancer pain?
The most appropriate dose is 10% to 15% of the total daily opioid dose as needed every 3 to 4 hours.
What is vitamin B12 a cofactor for?
Vitamin B12 is a cofactor for converting methylmalonic acid into succinyl-CoA, which then goes into the Krebs cycle.
How is a poorly differentiated neoplasm diagnosed?
A poorly differentiated neoplasm is diagnosed on fine-needle aspiration biopsy.
What is required for a core biopsy?
A core biopsy requires immunohistochemical analysis.
What do guidelines recommend for patients with liver cirrhosis?
Patients with liver cirrhosis should undergo surveillance ultrasound every 6 months, with or without alpha-fetoprotein testing.
What is the reticulocyte count when anemia is caused by marrow infiltration by CLL cells?
The reticulocyte count is low.
What should a metaphyseal fracture in a child or young adult prompt evaluation for?
It should prompt evaluation for an underlying cause such as a primary bone tumor.
What is the recommended surveillance testing for patients with smoldering multiple myeloma?
Patients should undergo surveillance testing 2 to 3 months after initial diagnosis and if stable, monitored every 4 to 6 months for one year.
What are signs of superior vena cava syndrome?
Facial swelling, cough, and mediastinal widening on chest radiograph are signs of superior vena cava syndrome.
What is the final step in a root cause analysis?
The final step is to develop an action plan for system-wide change.
What can desmopressin reverse?
Desmopressin can reverse platelet dysfunction rapidly but transiently.
What is Gaucher disease?
Gaucher disease is the most common lysosomal storage disease caused by a deficiency of the glucocerebrosidase enzyme.
What are the symptoms associated with TTP?
TTP is associated with thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
What causes Hemophilia B?
Hemophilia B is caused by a factor IX deficiency.
What condition is associated with tumor lysis syndrome?
Deficiency in uric acid levels >8 mg/dL despite aggressive volume expansion are candidates for treatment with rasburicase.
What are the symptoms associated with TTP?
TTP is associated with a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
What is the best laboratory test to diagnose adrenocorticotropic hormone-associated paraneoplastic syndrome?
An overnight dexamethasone suppression test or a 24-hour urinary free cortisol test.
What type of bladder cancer is associated with exposure to aromatic amine dyes?
Urothelial carcinoma.
What is the treatment of choice for patients with multiple brain metastases?
Whole-brain radiation plus glucocorticoid therapy.
What laboratory findings suggest autoimmune hemolytic anemia?
Evidence of hemolysis and peripheral spherocytosis.
What is the most common extracolonic type of cancer seen in women with Lynch syndrome?
Endometrial cancer.
What are other potential sites of malignancy associated with Lynch syndrome?
Stomach, small bowel, pancreas, hepatobiliary tract, ovaries, ureter, and renal pelvis.
Why must the mediastinum be assessed in a patient with NSCLC and lymphadenopathy?
It must be found free of tumor to proceed with curative resection.
What cancer is most typically associated with parathyroid hormone-related peptide-induced hypercalcemia?
Squamous-cell carcinoma of the lung.
What acute leukemia is very sensitive to all-trans retinoic acid?
Acute promyelocytic leukemia.
What happens to platelets after binding to vWF?
Platelets change their shape and release mediators that lead to activation of more platelets.
What is a common finding in a patient with hypercalcemia?
Proteinuria.
What can lead to the activation of more platelets?
Hypercalcemia, proteinuria, anemia, and kidney disease can lead to the activation of more platelets.
What should be evaluated in a patient with hypercalcemia, proteinuria, anemia, and kidney disease?
Evaluate for the diagnosis of multiple myeloma.
What long-term medications are associated with low levels of vitamin B12?
Long-term use of metformin or a proton pump inhibitor has been associated with development of low levels of vitamin B12.
When can prophylactic transfusion be considered?
Prophylactic transfusion can be considered in patients whose platelet count drops below 10,000 per mm3 since the risk of spontaneous bleeding is high below this level.
Does a warfarin overdose typically lead to persistent coagulopathy?
No, a warfarin overdose does not typically lead to persistent coagulopathy.
What should reverse warfarin therapy quickly?
Vitamin K should reverse warfarin therapy pretty quickly.
What is indicative of a diagnosis of a superior pulmonary sulcus (Pancoast) tumor?
The gradual development of arm weakness and Horner syndrome in an older patient with a history of cigarette smoking is most indicative of a diagnosis of a superior pulmonary sulcus (Pancoast) tumor.
What happens in a mixing study if the prolonged aPTT is caused by a lack of clotting factors?
The factors contained in the normal plasma will normalize the aPTT.
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
ITP patients who relapse or are unresponsive to dexamethasone may respond to intravenous immunoglobulin.
What can stimulate platelet production in ITP patients?
Eltrombopag or romiplostim can stimulate platelet production in ITP patients.
What is reserved for patients refractory to medical management?
Splenectomy is reserved for patients refractory to medical management.
What should patients with essential thrombocythemia and risk factors for complications be treated with?
Patients with essential thrombocythemia who have risk factors for complications should be treated with cytoreductive therapy with hydroxyurea.
How is immune thrombocytopenia (ITP) in people with HIV initially treated?
ITP in people with HIV is initially treated with antiretroviral therapy.
What treatments are added if ITP persists after virologic suppression?
If ITP persists after virologic suppression, other treatments such as glucocorticoids are added.
What is Von Hippel–Lindau disease?
Von Hippel–Lindau disease is a rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma and hemangioblastoma of the central nervous system.
What is pheochromocytoma?
A tumor of the central nervous system.
What type of carcinoma is associated with clear-cell?
Clear-cell renal-cell carcinoma.
What occurs after platelet aggregation?
Formation of a white thrombus composed of platelets and fibrinogen.
What is TTP associated with?
A pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
What is the most appropriate management for a patient taking apixaban with life-threatening bleeding?
Reversal with andexanet alfa or prothrombin complex concentrate.
In what clinical settings is reversal of anticoagulant recommended?
- Bleeding at a critical site where there is a high risk that the function of a vital organ will be compromised with resulting disability or death.
- Bleeding that is life-threatening.
- Major bleeding that fails to respond to initial control measures.
What are critical bleeding sites?
Intracranial, thoracic, airway, pericardial, intra-abdominal (nongastrointestinal), retroperitoneal, intra-articular, and intramuscular bleeding.
What is the 4Ts score for HIT?
It takes into account the degree of thrombocytopenia, the timing of the decline in platelet count, the development of thrombosis or other sequelae, and the presence of other causes for thrombocytopenia.
What characterizes the 5q minus subtype of myelodysplastic syndrome?
Chronic transfusion-dependent anemia, which can lead to iron overload.
What is Epstein-Barr virus infection associated with?
Burkitt lymphoma, Hodgkin lymphoma, and nasopharyngeal carcinoma.
What are the clinical manifestations of PCT limited to?
The skin and liver.
What is Felty syndrome?
A condition that develops in about 1% of patients with rheumatoid arthritis, characterized by rheumatoid arthritis, splenomegaly, and neutropenia.
What lab findings are associated with anemia of chronic disease?
High ferritin level, low-normal iron level, and low total iron-binding capacity.
What is the likely diagnosis for anemia of chronic disease?
Anemia of chronic disease.
What is the goal of treatment for early-stage breast cancer?
To reduce local recurrence and breast cancer mortality among young women.
What is the treatment plan for young women with early-stage breast cancer treated with breast-conserving surgery?
The treatment plan should generally include irradiation of the breast.
What is the blast count for patients with accelerated-phase CML?
Patients have a blast count of 10% to 20%.
What is the blast count for patients with CML in blast crisis?
Patients have >20% blasts in the peripheral blood or bone marrow.
What is the first-line treatment for the 5q minus subtype of myelodysplastic syndrome?
The first-line treatment is lenalidomide.
What must be ruled out in essential thrombocythemia with a platelet count ≥1,000,000 per mm3?
Acquired von Willebrand syndrome must first be ruled out by measurement of ristocetin cofactor activity.
How does the left gonadal vein drain?
The left gonadal vein drains at a right angle into the left renal vein.
How does the right gonadal vein insert?
The right gonadal vein inserts directly into the inferior vena cava at a more acute angle, which makes drainage easier.
What is the diagnosis for leukocytosis above 100,000 cells per mm3 with absolute basophilia, thrombocytosis, and splenomegaly?
The most likely diagnosis is chronic myeloid leukemia.
What test is used to evaluate for von Willebrand disease brought on by aortic stenosis?
Von Willebrand multimer analysis.
What is the treatment for radiation pneumonitis?
The treatment is glucocorticoid therapy, usually consisting of a 2-week course of prednisone followed by a gradual taper.
What is the treatment for lobular carcinoma in-situ?
The treatment is surgical excision followed by tamoxifen administration.
What is the risk category for patients with acute lymphocytic leukemia and a leukocyte count >50,000 cells per mm3?
They are in the highest risk category for TLS.
What is squamous-cell carcinoma of the oropharynx associated with in a non-smoker?
It is most often associated with human papillomavirus infection, particularly HPV 16.
What is the most appropriate management approach for chronic immune thrombocytopenia in a patient with persistent asymptomatic moderate thrombocytopenia after splenectomy?
The approach is observation with no additional treatment.
How is asymptomatic localized Kaposi sarcoma managed?
It is managed by close observation after initiation of antiretroviral therapy.
What are patients who have undergone gastric bypass surgery at risk for?
They are at risk for iron deficiency.
What deficiencies are patients at risk for after gastric bypass surgery?
Patients who have undergone gastric bypass surgery are at risk for iron deficiency and vitamin B12 deficiency.
What develops as food bypasses iron absorption sites in the duodenum?
Iron deficiency anemia develops as food bypasses iron absorption sites in the duodenum.
What is the most appropriate approach to managing intractable back pain in a patient with metastatic cancer?
The most appropriate approach is to initiate bolus doses of intravenous opioids.
What symptoms are commonly associated with taxanes such as paclitaxel?
Taxanes such as paclitaxel are commonly associated with sensory neuropathy.
What characterizes TTP?
TTP is characterized by deficiency of ADAMTS13, a plasma protease that normally cleaves ultra-large von Willebrand factor multimers.
What is the most appropriate dose of short-acting opioids for breakthrough pain in patients with uncontrolled cancer pain?
The most appropriate dose is 10% to 15% of the total daily opioid dose, as needed every 3 to 4 hours.
What are topoisomerase inhibitors and give an example?
Topoisomerase inhibitors are chemotherapy agents such as doxorubicin.
What condition can lead to leukemia?
Leukemia can occur even earlier after treatment.
What characterizes reversible coagulopathy?
It is characterized by a high international normalized ratio and deficiencies of factors II, VII, IX, and X.
What should raise suspicion for superwarfarin intoxication?
Deficiencies of factors II, VII, IX, and X should raise suspicion for superwarfarin intoxication.
What are Auer rods associated with?
Auer rods are associated with disseminated intravascular coagulation and the chromosomal translocation t(15;17), which is acute promyelocytic leukemia.
What is the most likely diagnosis in a young man with severe anemia?
The most likely diagnosis is acquired pure red-cell aplasia.
What findings are associated with acquired pure red-cell aplasia?
Normal platelets and white blood cells, a low reticulocyte count, and a lack of erythroid precursors on bone-marrow examination.
What is the treatment for acquired pure red-cell aplasia?
Treatment generally involves immunosuppression.
What does PNH lead to?
PNH leads to the partial or complete absence of glycosylphosphatidylinositol-linked proteins, e.g., CD55 and CD59.
What is Von Hippel–Lindau disease?
Von Hippel–Lindau disease is a rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma of the central nervous system, pheochromocytoma, and clear-cell renal-cell carcinoma.
What does the diagnostic workup for squamous-cell carcinoma of the head and neck include?
It includes direct laryngoscopy, examination under anesthesia, and concurrent bilateral tonsillectomy.
What can lead to end-organ dysfunction?
Iron overload from transfusion-related etiologies can lead to end-organ dysfunction, especially in the liver, myocardium, and endocrine organs.
What are PNH cells susceptible to?
PNH cells are susceptible to complement-mediated lysis that occurs in the intravascular space and causes hemoglobinuria and hemosiderinuria.
What is G6PD deficiency?
G6PD deficiency is an X-linked recessive disease.
What is the best initial treatment for warm autoimmune hemolytic anemia?
The best initial treatment is a glucocorticoid.
What is W251bGwsIlpjMmUxZDJmODJlZWFmNTE0NjdjMmFkMjFiMjJhMGZjMCJd?
It is a glucocorticoid.
What should be considered with glucocorticoid therapy?
Concurrent or early initiation of rituximab.
What is a transfusion reaction?
It is defined as fever >38°C or an increase of at least 1°C from baseline that occurs during or up to 4 hours after a blood transfusion.
What is the treatment of choice for patients with multiple brain metastases?
Whole-brain radiation plus glucocorticoid therapy.
What is the follow-up for low-risk MGUS laboratory evaluation?
Labs can be repeated in approximately 3 months and then at 6- to 12-months.
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim–sulfamethoxazole, quinine, vancomycin, and penicillin.
What causes Type 2N von Willebrand disease?
Mutations in von Willebrand factor that affect the binding site for factor VIII.
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
Beta-thalassemia trait.
What type of cancer is most associated with acanthosis nigricans?
Gastric cancer.
How does radiation-associated angiosarcoma typically manifest?
It manifests several years after therapy with a cutaneous tumor that looks similar to a bruise or hematoma.
What is the prognosis for patients with radiation-associated angiosarcoma?
Prognosis is usually poor as local and distant recurrence is common.
What is the risk for developing Kaposi sarcoma in patients with human herpesvirus 8 seropositivity?
They have a high risk if they undergo transplantation and immunosuppression.
What is the risk for cervical cancer in people with HIV?
It is much higher than in the general population, even in patients taking antiretroviral therapy.
What is the most likely diagnosis in a young man with HIV?
A rapidly progressive condition.
What is the most likely diagnosis in a young man with HIV presenting with a rapidly enlarging neck mass?
Burkitt lymphoma
What symptoms are associated with Burkitt lymphoma?
Night sweats and weight loss
What results from hereditary spherocytosis?
Diminished compliance of the cell and degradation by the spleen resulting in splenomegaly, anemia, and jaundice.
What does the diagnostic workup for squamous-cell carcinoma of the head and neck include?
Direct laryngoscopy, examination under anesthesia, and concurrent bilateral tonsillectomy.
What is the most appropriate initial therapy for a patient with newly diagnosed thrombotic thrombocytopenic purpura?
Therapeutic plasma exchange
What can copper deficiency manifest as?
Pancytopenia with macrocytic anemia
What should be considered as a likely diagnosis in a patient with urticaria pigmentosa and anaphylaxis in response to an antibiotic?
Systemic mastocytosis
What is a typical manifestation of renal-cell carcinoma?
Hematuria, flank pain, or a palpable abdominal mass
What is chronic HIV infection a well-characterized cause of?
Immune thrombocytopenic purpura (ITP)
What does the 4Ts score for HIT take into account?
- Degree of thrombocytopenia, 2. Timing of the decline in platelet count, 3. Development of thrombosis or other sequelae, 4. Presence of other causes for thrombocytopenia.
What occurs after platelet aggregation?
Formation of a white thrombus composed of platelets and fibrinogen.
What are often present in chronic myeloid leukemia (CML)?
Thrombocytosis and splenomegaly
What is the most common initial test for HIT?
An enzyme-linked immunosorbent immunoassay (ELISA) that identifies the presence of antiplatelet factor 4 antibodies in serum.
What are the effects of tamoxifen?
Antiestrogenic effects in the breast and estrogenic effects in the uterus and bone, which may cause hyperplasia and worsen cancer.
What is the appropriate treatment to relieve symptoms of diarrhea and flushing associated with carcinoid syndrome?
A somatostatin analogue such as octreotide while controlling tumor growth.
What is marked anemia?
A significant decrease in red blood cells or hemoglobin levels.
What medication is associated with tumor lysis syndrome?
Octreotide
What does the presence of spherocytes in a patient with SLE suggest?
Autoimmune hemolytic anemia
What uric acid level indicates the need for treatment with rasburicase?
Uric acid levels >8 mg/dL despite aggressive volume expansion
Is amyloidosis associated with massive hepatosplenomegaly?
Amyloidosis is not associated with massive hepatosplenomegaly
Where do most CNS lymphomas cluster on imaging?
Supratentorially with a predilection for the deep gray matter of the frontal and parietal lobes
What should a fracture in a long bone of a young adult prompt evaluation for?
Osteosarcoma
What is the most common primary bone tumor affecting children and young adults?
Ewing sarcoma
What is the recommended screening for people aged 50 to 80 with a history of smoking?
Annual screening with low-dose chest CT
What are major clinical manifestations of mixed cryoglobulinemic vasculitis?
Palpable purpura, kidney disease, arthralgia, myalgia, peripheral neuropathy
At what age can screening mammography be discontinued?
Approximately 75 years of age
What is typical of chronic lymphocytic leukemia?
Peripheral lymphocytosis with small lymphocytes
Does early treatment of asymptomatic patients with CLL improve outcomes?
No, it does not improve outcomes
What are patients with lymphoma at risk for?
Developing a rapidly enlarging mediastinal mass with airway obstruction
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III)
What is CLL characterized by?
CLL has an increase in mature appearing B lymphocytes.
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III) activates factor VII.
What are meningiomas?
Meningiomas are diffusely enhancing lesions that occur most commonly in the parasellar regions of the base of the skull or cerebral convexities.
Where do most CNS lymphomas cluster?
Most CNS lymphomas cluster around the ventricles with a predilection for the deep gray matter of the frontal and parietal lobes.
What is the most likely diagnosis in a patient with pancytopenia without myeloid immaturity?
The most likely diagnosis is Gaucher disease.
What is Gaucher disease?
Gaucher disease is the most common lysosomal storage disease, an autosomal recessive disorder caused by a deficiency of the glucocerebrosidase enzyme, leading to the accumulation of glycolipids.
Why is iron deficiency common in dialysis patients?
Iron deficiency is common in dialysis patients because of frequent blood testing and blood lost from the procedure.
Are carcinoid tumors typically associated with urticarial skin lesions?
Carcinoid tumors are not typically associated with urticarial skin lesions.
What are the functional assays that test the ability of a patient’s HIT antibodies?
The heparin-induced platelet aggregation (HIPA) assay and the serotonin release assay (SRA) are functional assays that test the ability of a patient’s HIT antibodies to activate test platelets.
What is the first-line therapy for hemolytic PNH?
The first-line therapy for hemolytic PNH is eculizumab, a monoclonal antibody.
What is a monoclonal antibody?
A monoclonal antibody is an antibody produced from a single clone of cells, designed to target a specific antigen.
What does a monoclonal antibody target?
It targets complement protein C5.
What is included in the appropriate evaluation of adenocarcinoma in an axillary lymph node?
Bilateral diagnostic mammography with or without ultrasound of the breast.
What does lupus anticoagulant lead to?
It leads to a prolonged prothrombin time or partial-thromboplastin time.
What is the most likely cause of a new-onset bleeding disorder in a previously healthy postpartum woman?
Acquired factor VIII inhibitor.
What is the role of Factor VIIa and tissue factor?
They form a complex that requires factor IV for calcium.
What is the best initial treatment for warm autoimmune hemolytic anemia?
A glucocorticoid with consideration for concurrent or early initiation of rituximab.
What causes cold agglutinin hemolytic anemia?
IgM antibodies bind to polysaccharides on erythrocytes.
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim-sulfamethoxazole, quinine, vancomycin, and penicillin.
What can benefit a patient with kidney failure and hyperuremia?
Desmopressin administration can increase vWF-factor 8 multimers.
What is indicated by leukocytosis above 100,000 cells per mm3?
It may be associated with absolute basophilia and thrombocytosis.
What is the cell count indicating absolute basophilia?
100,000 cells per mm3
What are common findings in chronic myeloid leukemia?
Thrombocytosis and splenomegaly
What is the most likely diagnosis with thrombocytosis and splenomegaly?
Chronic myeloid leukemia
What infections can cause cold agglutinin hemolytic anemia?
Mycoplasma or Epstein–Barr virus
What is Von Hippel–Lindau disease?
A rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma, pheochromocytoma, and clear-cell renal-cell carcinoma.
What is the most common cause of death in patients with PNH?
Thrombosis
What condition can some patients with PNH experience?
Bone-marrow failure resulting in pancytopenia
What is PNH associated with?
Increased risk for myelodysplastic syndrome, aplastic anemia, and leukemia.
What percentage of GISTs have mutations in proto-oncogene KIT?
Approximately 80%
What is Burkitt lymphoma?
An aggressive non-Hodgkin lymphoma associated with c-myc translocation usually in association with Epstein–Barr virus infection.
What factors are thought to precipitate PCT?
Multiple risk factors contributing to iron accumulation and/or oxidative stress in hepatocytes.
How can renal-cell carcinoma typically manifest?
As hematuria, flank pain, or a palpable abdominal mass.
What is the most appropriate medication for preventing thromboembolism in a patient with nonvalvular atrial fibrillation?
Not specified in the provided text.
What are risk factors associated with chronic kidney disease?
Warfarin is a risk factor associated with chronic kidney disease.
What can radiotherapy for prostate cancer lead to?
Radiotherapy for prostate cancer can lead to delayed bowel injury known as chronic radiation proctitis.
When does vancomycin-induced thrombocytopenia often start?
Vancomycin-induced thrombocytopenia often starts after around 5 days of treatment with a mean nadir platelet count of 13.
What is the mean nadir platelet count in vancomycin-induced thrombocytopenia?
The mean nadir platelet count is 600 per mm3 occurring 8 days after initiation of treatment.
Can granulocyte colony-stimulating factor be given to patients with CLL?
Granulocyte colony-stimulating factor can be given to patients with CLL if they also have neutropenia.
What is the most appropriate first-line treatment for stroke prevention in a patient with sickle cell disease?
The most appropriate first-line treatment for stroke prevention in a patient with sickle cell disease and a history of transient ischemic attacks is not specified.
What is prophylactic red-cell exchange transfusions?
Prophylactic red-cell exchange transfusions are a medical procedure used to prevent complications in patients.
No back content.
What does a mixing study reveal if the prolonged aPTT is caused by a lack of clotting factors?
In a mixing study, if the prolonged aPTT is caused by a lack of clotting factors, the factors contained in the normal plasma will normalize the aPTT.
No back content.
What is celiac plexus neurolysis?
Celiac plexus neurolysis is a percutaneous or endoscopic ultrasound-guided method for achieving pain control.
No back content.
What is the purpose of celiac plexus neurolysis?
It is used for pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
The organs of the upper abdomen typically send nociceptive pain signals through the celiac plexus to the spinal cord.
What substances are injected during celiac plexus neurolysis?
During celiac plexus neurolysis, alcohol or phenol is injected to destroy the nerve tissue.
This reduces the transmission of pain signals.
What percentage of patients with pain from pancreatic cancer experience significant improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
Although most patients continue to experience some pain.
What is thrombotic thrombocytopenic purpura classically associated with?
Thrombotic thrombocytopenic purpura is classically associated with microangiopathy, thrombocytopenia, renal insufficiency, mental status changes, and fever.
No back content.
What are the first-line treatments for symptomatic anemia in patients with low-risk myelodysplastic syndrome?
The most appropriate first-line treatments are erythropoietin and transfusion support.
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What is the classic appearance of meningiomas on imaging?
Meningiomas are adjacent to bone and anchored to the dura, rendering the classic appearance of the ‘dural tail’ on imaging.
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Are adults with mild sickle cell disease likely to have splenomegaly?
Adults with even mild sickle cell disease are unlikely to have splenomegaly because they experience splenic infarcts during crises.
The splenomegaly is an important hint to the presence of SC disease.
What are the symptoms of acute porphyria?
Symptoms include severe abdominal pain, sensory and peripheral neuropathies, neuropsychiatric abnormalities, and red-tinged urine.
Diagnostic Test: urine test for porphobilinogen.
What medications will precipitate acute porphyria?
Sulfonamides, barbiturates, and hydantoins (phenytoin) will precipitate acute porphyria.
No back content.
What is neurolysis?
Neurolysis is a percutaneous or endoscopic ultrasound-guided method for achieving pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
How do the organs of the upper abdomen send pain signals?
The organs of the upper abdomen typically send nociceptive pain signals through the celiac plexus to the spinal cord.
What substances are injected during celiac plexus neurolysis?
During celiac plexus neurolysis, alcohol or phenol is injected, destroying the nerve tissue and thus reducing transmission of pain signals.
What percentage of patients with pain from pancreatic cancer experience significant improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
What is transfusion-associated lung injury?
Transfusion-associated lung injury usually manifests 1 to 6 hours after the transfusion is initiated.
In which individuals does chronic lymphocytic leukemia (CLL) occur more frequently?
Chronic lymphocytic leukemia (CLL) occurs more frequently in older individuals.
What indicates functional iron deficiency in patients with chronic kidney failure?
Functional iron deficiency is likely in patients with chronic kidney failure if they have a ferritin level of ≤500 ng/mL or a transferrin saturation of ≤30%.
How should patients with chronic lymphocytic leukemia and serious recurrent infections be treated?
Patients with chronic lymphocytic leukemia and serious recurrent infections should be treated with intravenous immunoglobulin.
What is hereditary hemochromatosis (HH) commonly caused by?
Hereditary hemochromatosis (HH) is most commonly caused by mutations in the HFE gene, such as C282Y or less commonly H63D.
What are typical manifestations of renal-cell carcinoma?
Renal-cell carcinoma can typically manifest as hematuria, flank pain, or a palpable abdominal mass.
When should first-degree relatives of patients who develop colorectal cancer start screening?
First-degree relatives of patients who develop colorectal cancer before age 60 should start screening at either age 40 or 10 years before the age at which the index case was diagnosed, whichever comes first.
What are major clinical manifestations of mixed cryoglobulinemic vasculitis?
Major clinical manifestations of mixed cryoglobulinemic vasculitis include palpable purpura.
What is palpable purpura?
Palpable purpura is a type of skin rash characterized by raised purple spots.
Example: It can be associated with various conditions, including vasculitis.
What conditions can kidney disease lead to?
Kidney disease can lead to arthralgia and myalgia, as well as peripheral neuropathy.
What is hypocomplementemia?
Hypocomplementemia is a condition where complement levels in the blood are low.
Example: It can be associated with certain autoimmune diseases.
What are common causes of paraneoplastic syndrome?
Bronchial carcinoid tumors are among the most common causes of paraneoplastic syndrome.
What is Cushing syndrome?
Cushing syndrome is a condition caused by excess cortisol in the body.
What is therapy-related acute myeloid leukemia?
Therapy-related acute myeloid leukemia classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
What does a periosteal reaction indicate?
A periosteal reaction is indicative of Ewing sarcoma.
What is the management for life-threatening bleeding in patients taking apixaban?
The management includes reversal with andexanet alfa or prothrombin complex concentrate.
What is celiac plexus neurolysis?
Celiac plexus neurolysis is a method for achieving pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
What is the purpose of injecting alcohol or phenol during celiac plexus neurolysis?
Injecting alcohol or phenol destroys nerve tissue, thus reducing the transmission of pain signals.
What percentage of patients with pain from pancreatic cancer experience improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
What is the best approach to breast cancer screening for young women with a high lifetime risk?
The best approach is annual MRI in addition to annual mammography.
Why should both MRI and mammography be performed annually?
Both screening tests should be performed annually as MRI has better sensitivity in young women.
What imaging method has better sensitivity in young women with increased mammographic density?
Mammography is superior to MRI in detecting microcalcifications associated with ductal carcinoma in situ.
What is associated with reductions in bone mineral density during androgen-deprivation therapy?
Reductions can occur as early as the first 6 to 12 months of treatment.
What is the most prominent clinical finding of thrombocytopenia?
The presence of petechiae.
In what percentage of patients with cryoglobulin vasculitis is the disorder associated with chronic hepatitis C virus infection?
About 70%.
What are other causes of cryoglobulin vasculitis?
Other viruses, including hepatitis B virus, Epstein–Barr virus, or HIV.
What is the most likely diagnosis in a patient with pancytopenia and Auer rods?
Acute promyelocytic leukemia.
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III).
What anticoagulants have the most evidence to support their efficacy in preventing recurrent venous thromboembolism in cancer patients?
Low-molecular-weight heparin and direct oral factor Xa inhibitors.
What is characteristic of hairy-cell leukemia?
Pancytopenia in association with splenomegaly, B symptoms, monocytopenia, and atypical lymphocytes with projections.
What should be considered as a likely diagnosis in a patient with both urticaria pigmentosa and anaphylaxis in response to an antibiotic?
Systemic mastocytosis.
Why is thrombocytosis often seen in beta thalassemia patients?
Due to increased drive on the marrow from erythropoietin.
What is the most appropriate treatment for patients with chemotherapy-induced nausea and vomiting who do not respond to preventive treatment with prochlorperazine?
Intravenous palonosetron and dexamethasone.
What is the appropriate treatment for 3NSJd?
Intravenous palonosetron and dexamethasone.
What are the clinical manifestations of PCT limited to?
The skin and liver.
What percentage of GISTs have mutations in the proto-oncogene KIT?
Approximately 80%.
What type of skeletal metastases are predominant in certain cancers?
Osteoblastic or sclerotic skeletal metastases.
What should raise suspicion for metastatic prostate cancer?
Adenocarcinoma of an unknown primary site in a man.
What is the best initial management for patients with metastatic cancer admitted with intractable pain?
A patient-controlled analgesia pump with an intravenous opioid.
What features are associated with increased risk for progression of MGUS?
- A serum monoclonal protein level >1.5 g/dL
- Non–immunoglobulin G monoclonal protein
- A markedly abnormal free-light-chain-kappa-to-lambda ratio.
What is the best strategy for managing cancer-related pain that is intractable despite intermittent opioids?
Intravenous opioids administered as patient-controlled analgesia.
What is the continuous infusion rate for patient-controlled analgesia?
50% to 100% of the patient’s 24-hour opioid intake.
What is Porphyria cutanea tarda (PCT) caused by?
An acquired deficiency of hepatic uroporphyrinogen decarboxylase.
What are myelocytes precursors to?
Basophils, eosinophils, and neutrophils.
What does PNH lead to?
The partial or complete absence of glycosylphosphatidylinositol-linked proteins, e.g., CD55 and CD59.
What is the most effective management option for symptomatic progressive prostate cancer in older men with a life expectancy of <10 years?
Radiation therapy with consideration for androgen-deprivation therapy.
How can the risk of progression from asymptomatic to symptomatic MM be estimated?
Using the Mayo Clinic 2018 risk stratification system.
What are the three risk factors considered in the Mayo Clinic 2018 risk stratification system?
- M protein >2 g/dL
- Bone marrow.
What is the M protein level indicative of?
M protein >2 g/dL
What percentage of clonal plasma cells in the bone marrow indicates a condition?
Bone marrow clonal plasma cells >20%
What is the involved–uninvolved free-light-chain ratio threshold?
Involved–uninvolved free-light-chain ratio >20
What risk factors categorize patients with smoldering MM?
Patients are considered to be at low risk for smoldering MM if they have no risk factors, intermediate risk if they have one risk factor, or high risk if they have 2 or 3 risk factors.
What is the initial treatment for immune thrombocytopenia (ITP) in people with HIV?
ITP is initially treated with antiretroviral therapy.
What treatments are added if ITP persists after virologic suppression?
Other treatments such as glucocorticoids are added.
What is the first-line medication for reducing acute pain episodes in sickle cell disease?
The first-line medication is hydroxyurea.
Why are varicoceles common on the left side?
Varicoceles are fairly common on the left side because of the higher pressure within the left gonadal vein compared with the right gonadal vein.
What is the best screening for a patient with a sibling who has a history of acute myeloid leukemia?
The best screening is routine medical care.
How is acute chest syndrome defined?
Acute chest syndrome is defined as chest pain associated with a new opacity on chest radiography and associated fever or respiratory distress.
What is the risk of progression from asymptomatic to symptomatic MM estimated by?
The Mayo Clinic 2018 risk stratification system.
What are the three risk factors considered in the Mayo Clinic risk stratification for MM?
- M protein >2 g/dL
- Bone marrow clonal plasma cells >20%
- Involved–uninvolved free-light-chain ratio >20
What risk level are patients considered if they have no risk factors for smoldering MM?
Low risk.
What risk level are patients considered if they have one risk factor for smoldering MM?
Intermediate risk.
What risk level are patients considered if they have 2 or 3 risk factors for smoldering MM?
High risk.
What is likely the diagnosis for severe thrombocytopenia in a patient with no signs of bruising or bleeding?
Pseudothrombocytopenia.
What causes pseudothrombocytopenia?
Naturally occurring antibodies directed against an epitope on glycoprotein IIb/IIIa.
What anticoagulant should not be discontinued in patients at risk for recurrent thrombosis?
Warfarin.
What is the best strategy to manage warfarin in a patient needing a minor procedure?
Continue the anticoagulant therapy.
What are examples of minor procedures where anticoagulant therapy can be continued?
Tooth extraction, excision of basal- or squamous-cell carcinoma, and cataract surgery.
What is Von Hippel–Lindau disease?
A rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma of the central nervous system, pheochromocytoma, and clear-cell renal-cell carcinoma.
What do current guidelines recommend for screening for lung cancer?
Annual screening with low-dose chest CT for people age 50 to 80 years with a history of at least 20 pack-years of cigarette smoking.
What is the most appropriate treatment for a single small (<3.5 cm) brain metastasis in a patient with unresectable non–small-cell lung cancer?
Stereotactic radiosurgery.
What initiates the extrinsic pathway of coagulation?
Factor VIIa.
What initiates the extrinsic pathway of coagulation?
Factor VIIa and tissue factor form a complex.
What effect does Bupropion have on seizures?
Bupropion can lower seizure threshold.
What process does methionine undergo?
Methionine converts into homocysteine, then into cystathionine. Vitamin B12 can be used to recycle it back into methionine.
What inheritance pattern do autosomal dominant diseases exhibit?
They have incomplete penetrance and may occasionally appear to skip a generation.
What are the diagnostic criteria for MGUS?
Monoclonal protein detected in serum at a level <3.0 g/dL; <10% plasma cells in bone marrow; absence of hypercalcemia, renal failure, anemia, and lytic bone lesions.
What is the best strategy for managing cancer-related pain?
Intravenous opioids administered as patient-controlled analgesia with a continuous infusion rate equal to 50% to 100% of the patient’s 24-hour opioid intake.
How does the left gonadal vein drain?
The left gonadal vein drains at a right angle into the left renal vein.
How should a warfarin-treated patient with an elevated INR >10 be managed?
Administer low-dose vitamin K by mouth and withhold warfarin.
Where do gastrointestinal stromal tumors (GIST) commonly occur?
GIST can occur throughout the gastrointestinal tract, most commonly in the stomach.
What is a potential complication of oral agents that inhibit tyrosine kinase activity?
Interstitial lung disease is an uncommon but potentially fatal complication.
What causes Type 2N von Willebrand disease?
It is caused by mutations in von Willebrand factor that affect the binding site for factor VIII, leading to impaired binding and rapid clearance of the factor.
What can lead to impaired binding and rapid clearance of a factor in a patient with hypercalcemia?
Proteinuria, anemia, and kidney disease
Evaluate for the diagnosis of multiple myeloma.
What is Beck triad associated with?
Pericardial effusion
It includes low systolic blood pressure, jugular venous distention, and muffled heart sounds.
What is the most likely cause of normocytic normochromic anemia in a 35-year-old woman with bradycardia and menometrorrhagia?
Hypothyroidism.
How can patients with superwarfarin intoxication be managed?
With fresh frozen plasma and vitamin K
Followed by several months of tapering vitamin K therapy until the agent is eliminated from the body.
What is beta-thalassemia?
A congenital hemolytic anemia caused by reduced or absent synthesis of the beta chains of hemoglobin.
What are the phenotypes of beta-thalassemia?
They vary considerably, including asymptomatic carrier state (beta-thalassemia minor), moderate anemia, and severe transfusion-dependent anemia (beta-thalassemia major).
What must be ruled out in essential thrombocythemia if the platelet count is ≥1,000,000 per mm3?
Acquired von Willebrand syndrome
Must be ruled out by measurement of ristocetin cofactor activity.
What are the major criteria for Polycythemia Vera (PV)?
- Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men) or elevated hematocrit (>48% in women; >49% in men).
- Bone-marrow biopsy consistent with PV.
- Presence of the JAK2 V617F or another similar mutation.
What is a minor criterion for Polycythemia Vera (PV)?
Serum erythropoietin level below the normal reference range.
What is the significance of levels below the normal reference range?
It indicates a condition that may require further investigation, especially in most CNS cases.
Where do lymphomas typically cluster in the brain?
Lymphomas are supratentorial and cluster around the ventricles with a predilection for the deep gray matter of the frontal and parietal lobes.
What does beta-thalassemia lead to?
Beta-thalassemia leads to abnormal erythrocytes that express procoagulant phospholipids, activated clotting factors, and activated platelets, all of which predispose patients to thrombosis.
What tumor marker is most likely elevated in a yolk sac tumor?
The tumor marker most likely to be elevated in a yolk sac tumor is alpha-fetoprotein.
Why isn’t Von Willebrand’s disease the likely diagnosis for a young woman with excessive bleeding gums post-partum?
Von Willebrand’s disease is inherited; the absence of family and past history of bleeding makes it unlikely.
What is the association of EBV with cancers?
EBV is associated with nasopharyngeal cancers, Burkitt lymphoma, and Hodgkin lymphoma.
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
The most likely diagnosis is beta-thalassemia trait.
What is the blast count in patients with accelerated-phase CML?
Patients with accelerated-phase CML have a blast count of 10% to 20%.
What characterizes patients with CML in blast crisis?
Patients with CML in blast crisis have more than 20% blasts in the peripheral blood or bone marrow.
What deficiencies are patients at risk for after gastric bypass surgery?
Patients who have undergone gastric bypass surgery are at risk for iron deficiency and vitamin B12 deficiency.
What causes vitamin B12 deficiency after gastric bypass?
Vitamin B12 deficiency develops because of inadequate binding by intrinsic factor after gastric bypass.
What causes macrocytic anemia?
G6PD-deficient erythrocytes in combination with high levels of oxidants cause a cross-linking of sulfhydryl groups on globin chains; this causes their denaturation and the formation of Heinz bodies.
What is tumor lysis syndrome (TLS)?
TLS is caused by the release of the contents of malignant cells into the bloodstream resulting in electrolyte abnormalities.
What are some electrolyte abnormalities associated with TLS?
These may include hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia.
What is the triad of the most prominent clinical features of paroxysmal nocturnal hemoglobinuria?
The triad includes hemolytic anemia, thrombosis, and pancytopenia.
What is the most appropriate initial management approach for an asymptomatic patient with early-stage chronic lymphocytic leukemia?
Observation.
What is the most appropriate test to confirm a suspected diagnosis of hepatocellular cancer in a patient with cirrhosis?
Dynamic contrast-enhanced MRI or multiphasic CT of the liver.
What is the treatment of TLS?
Treatment includes rasburicase, aggressive volume expansion with normal saline, and if needed, a loop diuretic to achieve a target urine output of around 150–200 mL/hour for most adults.
What are the characteristics of very early stages of cervical cancer?
They are often asymptomatic and squamous-cell carcinomas comprise about 80% of all cases.
When may platelet-specific antibody testing be considered?
It may be considered in patients who fail to respond to HLA-compatible or cross-matched platelets and in patients at risk for post-transfusion complications.
What are platelets associated with in patients at risk for post-transfusion purpura?
Platelets are associated with the risk of post-transfusion purpura.
What are the prominent clinical features of paroxysmal nocturnal hemoglobinuria?
The triad of the most prominent clinical features includes hemolytic anemia, thrombosis, and pancytopenia.
What is thrombotic thrombocytopenic purpura classically associated with?
Thrombotic thrombocytopenic purpura is classically associated with microangiopathy.
What are the symptoms of superior vena cava syndrome?
Symptoms include a mediastinal mass, mental status changes, and fever.
How is hairy cell leukemia diagnosed?
Diagnosis requires soluble CD25 in serum and surface expression of CD103.
What is the first step in managing a patient with superior vena cava syndrome?
The first step is a biopsy.
What mediates platelet aggregation?
Platelet aggregation is mediated by GpIIb/IIIa-receptor and fibrinogen.
What is elevated in folate deficiency?
Homocysteine level is elevated, but not the methylmalonic acid level.
What does the treatment of TLS include?
The treatment includes rasburicase and aggressive volume expansion with normal saline.
What is the target urine output for most adults in TLS management?
The target urine output is around 150–200 mL/hour.
What can help diagnose HIT?
Testing for platelet factor 4 antibodies can be helpful in diagnosing HIT.
At what age can screening mammography be discontinued?
Screening mammography can be discontinued at approximately 75 years of age.
Where are most CNS lymphomas located?
Most CNS lymphomas are supratentorial, clustering around the ventricles with a predilection for the deep gray matter.
What causes pseudothrombocytopenia?
Pseudothrombocytopenia is due to EDTA altered platelet autoantibodies resulting in clumping of platelets.
What suggests autoimmune hemolytic anemia?
Evidence of hemolysis and peripheral spherocytosis suggests autoimmune hemolytic anemia.
What is a typical presentation of cervical cancer?
Irregular bleeding exacerbated by sexual intercourse is a typical presentation.
What are the major clinical manifestations of mixed cryoglobulinemic vasculitis?
Major clinical manifestations include palpable purpura, kidney disease, arthralgia, myalgia, and peripheral neuropathy.
What is arthralgia?
Arthralgia refers to joint pain.
What is myalgia?
Myalgia refers to muscle pain.
What is peripheral neuropathy?
Peripheral neuropathy is a condition resulting from damage to the peripheral nerves.
What is hypocomplementemia?
Hypocomplementemia is characterized by low levels of complement proteins in the blood.
What is chronic lymphocytic leukemia (CLL)?
Chronic lymphocytic leukemia is characterized by an increase of mature-appearing B lymphocytes.
What is therapy-related acute myeloid leukemia?
Therapy-related acute myeloid leukemia classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
What are elevated levels of methylmalonic acid and homocysteine indicative of?
Elevated levels of methylmalonic acid and homocysteine are indicative of vitamin B12 deficiency.
What type of melanoma accounts for fewer than 5% of melanomas?
Acral lentiginous melanoma accounts for fewer than 5% of melanomas.
What is the most common type of melanoma in dark-skinned people?
Acral lentiginous melanoma is the most common type of melanoma in dark-skinned people.
Where is acral lentiginous melanoma typically found?
Acral lentiginous melanoma is found on the palms, soles, and occasionally in mucous membranes.
What is the initial therapy for heavy menstrual bleeding with hemodynamically significant hemorrhage?
The initial therapy is red-blood-cell transfusion.
What does the TF-FVIIa complex activate?
The TF-FVIIa complex activates factor X and factor IX.
What happens in the presence of a clotting inhibitor such as lupus anticoagulant?
The clotting time (aPTT) remains increased.
What triggers hemolytic anemia in patients with G6PD deficiency?
Hemolytic anemia is often triggered by exposure to medications.
What is polycythemia vera?
Polycythemia vera is a myeloproliferative neoplasm characterized by increased red-cell mass and elevated hemoglobin.
What symptoms are associated with polycythemia vera?
Symptoms include erythromelalgia (red, hot, painful extremities) and pruritus.
What is the role of von Willebrand factor (vWF) in platelet hemostasis?
After binding to vWF, platelets change their shape and release mediators that activate more platelets.
What should postmenopausal women with vaginal bleeding and a history of tamoxifen use undergo?
They should undergo evaluation with in-office endometrial biopsy.
What is the most appropriate management for severe abdominal pain in a patient with pancreatic cancer developing adverse effects from high doses of opioids?
The most appropriate management is neurolysis, such as a celiac plexus block.
What is neurolysis?
Neurolysis is a procedure such as a celiac plexus block.
What is a 24-hour urine sample used for?
A 24-hour urine sample for 5-hydroxyindoleacetic acid would be used to diagnose and monitor a carcinoid tumor.
How often should patients with smoldering multiple myeloma be monitored?
Patients whose smoldering MM is being managed with observation alone should undergo surveillance testing 2 to 3 months after initial diagnosis. If stable, the patient should be monitored every 4 to 6 months for one year.
What is the follow-up for stable patients with smoldering multiple myeloma?
Stable patients who remain asymptomatic without evidence of end-organ damage can then be followed every 6 to 12 months.
What is the most likely diagnosis in a person with HIV and thrombocytopenia?
Immune thrombocytopenia is the most likely diagnosis in a person with HIV whose peripheral-blood smear shows thrombocytopenia with a few large platelets.
When does heparin-induced thrombocytopenia usually occur?
HIT usually occurs 5 to 14 days after exposure (earlier in those with prior heparin exposure) and does not generally reduce platelet counts to below 10,000 per mm3.
What is the most appropriate next step for managing cancer pain that persists despite high doses of one opioid?
The most appropriate next step is to rotate to another opioid.
When does vancomycin-induced thrombocytopenia typically start?
Vancomycin-induced thrombocytopenia often starts after around 5 days of treatment, with a mean nadir platelet count of 13,600 per mm3 occurring 8 days after initiation of treatment.
What is the most likely diagnosis in an asymptomatic person with a mean corpuscular volume in the 60s?
The most likely diagnosis is beta-thalassemia trait or alpha-thalassemia trait.
What are the symptoms associated with TTP?
TTP is associated with a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
What is the recommended follow-up for a smoker with a single solid pulmonary nodule?
A follow-up low-dose CT scan of the chest in 6 months is recommended.
What levels are elevated in patients with vitamin B12 deficiency?
Methylmalonic acid and homocysteine levels are elevated in patients with vitamin B12 deficiency.
What suggests a diagnosis of hemoglobin SC disease?
The combination of mild anemia and infrequent pain crises in a patient with splenomegaly suggests a diagnosis of hemoglobin SC disease.
What is the best strategy to manage acute severe pain?
The best strategy to manage acute severe pain is not specified in the provided text.
What does a diagnosis of hemoglobin SC disease suggest?
It suggests a specific type of hemoglobinopathy.
What is the best strategy to manage acute severe pain at the end of life?
Bolus doses of intravenous opioids.
What is required for the diagnosis of systemic mastocytosis?
A bone biopsy.
What condition is indicated by hereditary hemochromatosis (HH)?
It is most commonly caused by mutations in the HFE gene.
What are the common mutations associated with hereditary hemochromatosis?
C282Y or less commonly H63D.
In which demographic does hereditary hemochromatosis typically manifest?
In middle-aged white men, often of Irish heritage.
What are the clinical features of hereditary hemochromatosis?
Gradually progressive adenopathy and splenomegaly.
What systemic symptoms are concerning for lymphoma?
Fatigue, fever, night sweats, and weight loss.
What should clinicians always exclude in febrile transfusion reactions?
Hemolysis.
What test should be performed to check for hemolysis in transfusion reactions?
A direct antiglobulin test.
What medication frequently causes neutropenia?
Ganciclovir.
What are the major criteria for Polycythemia Vera (PV)?
- Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men). 2. Elevated hematocrit (>48% in women; >49% in men). 3. Bone-marrow biopsy consistent with PV.
What is a minor criterion for Polycythemia Vera?
Serum erythropoietin level below the normal reference range.
How can TACO be distinguished from TRALI?
By the presence of hypertension and distended neck veins suggesting increased central venous pressure.
What is the increased risk of cancer in patients with Common Variable Immunodeficiency (CVID)?
Particularly non-Hodgkin lymphoma.
What is the most appropriate treatment for transfusion-induced iron overload with evidence of end-organ dysfunction?
Iron chelation therapy with a treatment such as deferasirox.
What are the most common acquired risk factors for Budd-Chiari syndrome?
Myeloproliferative neoplasms.
What is the typical treatment for radiation-associated angiosarcoma?
Mastectomy is preferred.
What is the typical treatment for Radiation-associated angiosarcoma?
Mastectomy is typically preferred.
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
Beta-thalassemia trait.
What is the most likely diagnosis in a patient with pancytopenia and Auer rods?
Acute promyelocytic leukemia.
What is the chromosomal translocation associated with acute promyelocytic leukemia?
t(15;17).
How can low-risk neutropenia with fever be managed?
It can be managed with ciprofloxacin and amoxicillin–clavulanate.
What should patients with sickle cell disease and moderate or severe acute chest syndrome receive?
They should receive short-term care consisting of red-cell exchange transfusion.
What test is used to confirm a suspected diagnosis of autoimmune hemolytic anemia?
A direct antiglobulin test.
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
They may respond to intravenous immunoglobulin stimulation of platelet production with eltrombopag or romiplostim.
What is the treatment for patients refractory to medical management?
Splenectomy is reserved for patients refractory to medical management.
What is a leading cause of death in adult patients with sickle cell disease?
Acute chest syndrome is a leading cause of death in adult patients with sickle cell disease.
When should positron emission tomography and electron microscopy be used?
They should be used only if the diagnosis is unclear after light microscopy and IHC.
What should patients with essential thrombocythemia and risk factors for complications be treated with?
They should be treated with cytoreductive therapy with hydroxyurea.
What does the 4Ts score for HIT take into account?
The 4Ts score takes into account the (1) degree of thrombocytopenia, (2) the timing of the decline in platelet count, (3) the development of thrombosis or other sequelae, and (4) the presence of other causes for thrombocytopenia.
What is squamous-cell carcinoma of the oropharynx associated with in non-smokers?
It is most often associated with human papillomavirus infection.
What forms a complex in the coagulation process?
Factor VIIa and tissue factor form a complex.
What is the mean age at diagnosis of renal-cell carcinoma in patients with von Hippel–Lindau disease?
The mean age at diagnosis is approximately 44 years.
What characterizes essential thrombocythemia?
It is characterized by excessive production of platelets in the absence of increased red-cell mass.
What is one of the minor diagnostic criteria for systemic mastocytosis?
A serum tryptase level >20 ng/mL in the absence of an associated myeloid disorder.
What is the recommended initial anticoagulant for uncomplicated venous thromboembolism?
The recommended initial anticoagulant is a DOAC.
What is a DOAC?
A DOAC is a direct oral anticoagulant that does not correct the activated partial-thromboplastin time in a mixing study, suggesting the patient’s plasma contains a factor that inhibits one of the clotting factors.
What is the most common factor inhibited in patients with a DOAC?
The most common factor inhibited is factor 8.
What treatment has been associated with significant improvements in overall survival among patients receiving carboplatin and paclitaxel for metastatic non-small-cell lung cancer?
Bevacizumab.
What is the most appropriate treatment for a patient with end-stage liver disease and low fibrinogen levels who is bleeding with hemoglobin greater than 7?
Cryoprecipitate.
What is the most appropriate diagnostic test for an older man with an easily palpable breast mass?
Core biopsy.
