Hematology/Oncology

Question 1

What are the lung cancer screening recommendations?

Answer 1

Annual low-dose CT scan screening for high risk individuals whoare; ages 55-74, with 30 pack year history or smoking who are a current smoker or quit within the past 15 years.

Question 2

What is the epidemiology of DLBCL?

Answer 2

• Most common histologic subtype of NHL
• 25% of cases of NHL
• 7 cases per 100,000 persons per year
• M:F – 55:45
• Median age – 64y

Question 3

What is the pathogenesis of DLBCL?

Answer 3

• Arises from a mature B cell – centroblasts or immunoblasts
• Can arise de novo or through transformation of many different types of low grade B cell lymphomas
  
  • CLL
  • Lymphoplasmacytic Lymphoma
  • Follicular Lymphoma
  • MALT Lymphoma
  • Splenic Marginal Zone Lymphoma
• DLBCL is an AIDS-defining malignancy
  
  • Characterized by more frequent B symptoms, extranodal disease, unusual location involvement, and advanced stage at diagnosis

Question 4

What is the clinical presentation of DLBCL?

Answer 4

Rapidly enlarging symptomatic mass, fever, weight loss, night sweats, elevated LDH.

At presentation

• 60% have advanced stage DLBCL (III or IV)
• 30% bone marrow involvement
• 40% arise in extranodal extramedullary tissues
  
  • Most commonly stomach/GI tract

Question 5

How do you diagnose DLBCL?

Answer 5

• Excisional lymph node biopsy
• Morphology
  
  • Large, transformed B cells with prominent nucleoli and basophilic cytoplasm
• Immunophenotyping
  
  • CD 19, CD 20, CD22, CD79a

Question 6

How do you treat DLBCL? What are some of the principles of chemotherapy?

Answer 6

• Limited stage (I/II) – can be contained within one irradiation field
  
  • 30-40% of patients with DLBCL
  • Treated with abbreviated systemic chemotherapy (3 cycles), rituximab and radiation therapy.
• Advanced stage (III/IV) – Cannot be contained within one irradiation field
  
  • 60-70% of patients with DLBCL
  • Treated with systemic chemotherapy plus rituximab
• Chemotherapy
  
  • R- Rituximab
  • C- Cyclophosphamide
  • H- Doxorubicin
  • O- Vincristine
  • P- Prednisilone

Question 7

What is the epidemiology of ALL?

Answer 7

• Peak prevalence between ages 2-5
• Incidence in adults: 6000 cases per year in USA (20% of adult acute leukemias)
• Risk factors – ionizing radiation, previous alkylating agents, chromosomal abnormalities

Question 8

What are the symptoms of ALL?

Answer 8

• Constitutional symptoms
• Bleeding/infection – DIC ~10% of acute presentations
• Fatigue
• Infection
• Shortness of breath, chest pain - leukostasis
• HA, confusion, blurred vision, cranial nerve palsy – leukostasis or CNS involvement possible

Question 9

How do you diagnose ALL?

Answer 9

• Peripheral blood smear
• Bone marrow biopsy – gold standard
  
  • 20% blast cells minimum
  • Flow cytometry - immunophenotyping
  • B cell (70%) vs T cell (30%)
  • Cytogenetics important for treatment/prognosis
• Lumbar puncture – rule out CNS involvement (high incidence of CNS involvement up to 40%)

Question 10

What is HLH?

Answer 10

Hemophagocytic lymphohistiocytosis is a rare disease (more common in children than adults) due to the over activation of macrophages. It can be primary (familial) or secondary (after a severe infection, auto-immune disease, rheumatologic disease, or malignancy).

Question 11

How do you diagnose HLH?

Answer 11

Presence of five of the eight following clinical criteria

1. Fever
2. Cytopenias in 2 of 3 lineages
3. Splenomegaly
4. Hypertriglyceridemia and/or hypofibrinogenemia
5. Hemophagocytosis
6. Low or absent NK cell activity
7. Hyperferritinemia
8. Elevated plasma levels of soluble CD25

Question 12

What is auto-immune hemolytic anemia? What are the two types of categories of auto-immune hemolytic anemia?

Answer 12

The mechanism underlying autoimmune hemolytic anemia (AIHA) involves antibodies binding to erythrocytes, with or without the activation of complement, leading to erythrocyte destruction.

Antibodies causing hemolysis can be divided into two categories, warm and cold.

• Antibodies reacting to erythrocytes at body temperature lead to warm autoimmune hemolytic anemia (WAIHA).
• Those reacting at less than normal body temperature lead to a form of autoimmune hemolytic anemia also termed cold agglutinin disease.

Question 13

How do you diagnose auto-immune hemolytic anemia once hemolytic anemia is suspected?

Answer 13

If you suspect auto-immune hemolytic anemia, need to perform a direct and indirect antiglobulin test

Question 14

How do you treat warm auto-immune hemolytic anemia?

Answer 14

First-line: steroids +/- Rituximab

• The recommended initial doses are quite high (1 to 1.5 mg/kg per day of prednisone)
• In adults, tapering is slow in an attempt to find the lowest dose that will maintain an adequate remission.

Second-line:

• Splenectomy and Rituximab

Third-line:

• Immunosuppressive or cytotoxic agents (eg, azathioprine, cyclophosphamide, cyclosporine)