Hematology & Oncology

Question 1

Features of multiple endocrine neoplasia type 1?

Answer 1

1. Pituitary adenomas
2. Parathyroid adenomas
3. Enteropancreatic tumors

Question 2

Laboratory hallmarks of tumor lysis syndrome

Answer 2

1. Hyperuricemia
2. Hyperkalemia
3. Hyperphosphatemia

Question 3

Primary immune thrombocytopenia pathophysiology, presentation & management

Answer 3

(formerly known as idiopathic thrombocytopenia purpura)

• Pathophysiology: anti-platelet Ab attack platelets –> peripheral platelet destruction
• Presentation: petechiae, gingival bleeding, epistaxis, menorrhagia, GI bleed, ICH (in children, history of recent viral infection)
• Management: supportive (children), corticosteroids & IVIG/anti-D immunoglobulin (adults); platelets (severe bleeding/extremely low platelet count) splenectomy (refractory cases), rituximab, thrombopoietin receptor agonist, immunosuppressants

Question 4

Thrombotic thrombocytopenia purpura pathophysiology, presentation & management

Answer 4

• Pathophysiology: severe ADAMTS13 deficiency 2/2 inhibitory auto-Abs
• Presentation: fever, MAHA, thrombocytopenia, AMS & renal failure
• Management: plasmapheresis

Question 5

Tumor lysis syndrome pathophysiology, presentation & management

Answer 5

• pathophysiology: death of numerous neoplastic cells releasing large quantities of intracellular content & uric acid (typically 1-5 days after chemotherapy) –> hyperuricemia, hyperkalemia, hyperphosphatemia & hypocalcemia
• presentation: fatigue/lethargy, N/V, cloudy urine, muscle spasms & AMS
• management: aggressive IV hydration (kidneys excrete uric acid, potassium & phosphate), allopurinol (prevents conversion of hypoxanthine to uric acid) or rasburicase (converts uric acid to inactive & soluble metabolite)

Question 6

Acute myeloid leukemia epidemiology, pathophysiology, presentation & prognosis

Answer 6

• epidemiology: most common leukemia in adults; 15-20% of childhood leukemias; median age of diagnosis 65 yr
• pathophysiology: clonal proliferation of myeloid precursor cells –> reduction in production of other cells (RBC’s, platelets, mature granulocytes)
• presentation: related to pancytopenia; pallor, fatigue, easy bruising, gingival bleeding, petechiae and infections; labs with normocytic normochromic anemia with Auer rods
• prognosis: 5-year survival rate 25%

Question 7

Acute lymphoblastic leukemia epidemiology, pathophysiology & presentation

Answer 7

• epidemiology: most common cancer in children (age 3-7) with bimodal distribution (65 yo)
• pathophysiology: proliferation of blast cells
• presentation: bone pain, LAD, hepatosplenomegaly, CNS involvement

Question 8

Chronic lymphocytic leukemia epidemiology, pathophysiology, presentation, management & prognosis

Answer 8

• epidemiology: male predominance; median age of diagnosis 70 yr; most common leukemia in adults of Western countries
• pathophysiology: monoclonal proliferation of incompetent mature B cells
• presentation: asymptomatic at diagnosis; B symptoms, LAD (painless), splenomegaly, anemia & thrombocytopenia; peripheral smear with isolated lymphocytosis & smudge cells (fragile lymphocytes)
• management: observation for early-stage or asymptomatic; purine analogs, antibodies, alkylating agents, IVIG (for hypogammaglobulinemia & frequent infection)
• prognosis: virtually incurable; median survival 4-20 years

Question 9

Chronic myeloid leukemia epidemiology, pathophysiology, presentation & management

Answer 9

• epidemiology: least common leukemia
• pathophysiology: Philadelphia chromosome –> neutrophilic leukocytosis
• presentation: most asymptomatic; fatigue/weight loss, night sweats, LUQ pain, early satiety, bone pain, hepatosplenomegaly
• management: allogenic hematopoietic stem cell transplant (only curative treatment), tyrosine kinase inhibitor, dasatinib, inferferon-alpha + cytarabine (greater toxicity)

Question 10

Neutropenic fever diagnostic criteria

Answer 10

Fever (single oral temp >38.3 C OR >38.0 C for >1 hr) + neutropenia (ANC <500 OR <1,000 w/ predicted nadir of <500 in 48 hr)

Question 11

Preferred AC for VTE in cancer patients

Answer 11

LMWH (cancer patients have variable responses to traditional vitamin K antagonist therapy)

Question 11

Preferred AC for VTE in cancer patients

Answer 11

LMWH (cancer patients have variable responses to traditional vitamin K antagonist therapy)

Question 12

Waldenstrom macroglobulinemia

Answer 12

• plasma cell dyscrasia leading to abnormal synthesis of IgM paraproteins
• most common cause of hyperviscosity syndrome

Question 12

Waldenstrom macroglobulinemia

Answer 12

• plasma cell dyscrasia leading to abnormal synthesis of IgM paraproteins
• most common cause of hyperviscosity syndrome