Chelsea Slade's Q set on Leukemia/Lymphoma!

Leukemias and Lymphomas! 

1.  What is the primary tumor-killing cell of the immune system?

            A)  CD4 cells

            B) macrophages

            C) CD8 cells

            D) NK cells

            E) plasma cells

            F) Chuck Norris

2.  Which statement accurately describes a mechanism used by tumor cells to evade elimination by the immune system?

            A) Tumor cells express more MHCI molecules, which lets them hide from T cells, but attracts NK cells

            B) Tumor cells express more MHCI molecules, which lets them hide from NK cells, but attracts T cells

            C) Tumor cells express less MHCI molecules, which lets them hide from T cells, but attracts NK cells

            D)  Tumor cells express less MHCI molecules, which lets them hide from NK cells, but attracts T cells

3-6.  Match the affected protein with the appropriate translocation:

            A) c-myc

            B) cyclin D1

            C) BCL2

            D) BCR-ABL

            3. t(14;18)

            4. t(8;14)

            5. t(11;14)

            6. t(9;22)

7.  Which of the following is not a type of B-cell NHL?

            A) Anaplastic large cell lymphoma

            B) Small lymphocytic lymphoma

            C) Marginal zone lymphoma

            D) Burkitt lymphoma

8.  Which type of lymphoma is most likely to exhibit a nodular growth pattern?

            A) HL

            B) Follicular lymphoma

            C) T-cell lymphoma

            D) B and C

9-16.  Match the lymphoma to its associated characteristic.  Each answer may be used once, more than once, or not at all:

            A) follicular lymphoma

            B) marginal zone lymphoma

            C) Burkitt lymphoma

            D) mycosis fungoides

            9. can be treated with antibiotics

            10. cerebriform nuclei

            11. c-myc

            12. made up of T-cells

            13. BCL-2

            14. t(14;18)

            15. EBV

            16. t(8;14)

17.  What is the most common type of NHL worldwide?

            A) follicular lymphoma

            B) marginal zone lymphoma

            C) Burkitt lymphoma

            D) diffuse large B-cell lymphoma

18.  Which type of cell is involved in Sezary syndrome?

            A) CD4 cells

            B) CD8 cells

            C) immature B cells

            D) mature B cells

19.  The Reed-Sternberg cell is an abnormal cell of which type?

            A) macrophage

            B) T cell

            C) B cell

20.  True or False: Hodgkin lymphoma is seen almost exclusively in elderly adults.

            A) true

            B) false

21.  Mr. Perkins, a 48yo man with hypertension and hyperlipidemia, is diagnosed with nodular sclerosing Hodgkin lymphoma.  Careful workup shows him to have involved nodes in the supraclavicular and inguinal nodes.  The spleen and bone marrow do not seem to be involved.  Mr. Perkins has not been experiencing any fever, night sweats, or weight loss, though he has had a chronic dry cough.  What stage lymphoma does Mr. Perkins have?

            A) Stage IA

            B) Stage IB

            C) Stage IIA

            D) Stage IIB

            E) Stage IIIA

            F) Stage IIIB

            G) Stage IVA

            H) Stage IVB

22.  True or False: plasma cell myelomas have a bimodal age distribution of incidence.

            A) true

            B) false

23.  Which of the following are common signs / symptoms of plasma cell myeloma?

            A) hypercalcemia

            B) proteinuria

            C) lytic bone lesions

            D) anemia

            E) infections

            F) two of the above

            G) three of the above

            H) four of the above

            I) all of the above

24.  Most common Ig secreted in monoclonal gammopathy?

            A) IgA

            B) IgD

            C) IgE

            D) IgG

            E) IgM

25.  Serum hyperviscosity is a common feature of which plasma cell dyscrasia?

            A) plasma cell myeloma

            B) Waldenstrom macroglobulinemia

            C) both

            D) neither

26.  Which leukemia is associated with the Philadephia chromosome?

            A) ALL

            B) AML

            C) CLL

            D) CML

27.  Which statement regarding leukemias is false?

            A) prognosis of acute leukemia is poor, except for childhood ALL

            B) proliferation of WBCs leads to reduced infections

            C) petechiae should prompt a workup for leukemia

            D) bone marrow biopsy is helpful for diagnosis

28.  True or false: APL carries a relatively favorable prognosis.

            A) true                         B) false

29. True or False: the leukocyte alkaline phosphatase score is increased in CML.

            A) true                         B) false

30.  Which leukemia will often show increased eosnophils and basophils in the bone marrow?

            A) ALL

            B) AML

            C) CLL

            D) CML

31.  True or false: CML can undergo an acute blast crisis and transform into either AML or ALL.

            A) true

            B) false

32.  Which of the following are features of polycythemia vera:

            A) increased EPO

            B) JAK2 mutation

            C) hyperviscosity

            D) two of the above

            E) all of the above

33.  Which of the following is primary myelofibrosis most likely to become if it undergoes a blast crisis?

            A) ALL

            B) AML

            C) equally likely to become ALL or AML

34.  Which of the following is not a feature of hairy cell leukemia?

            A) TRAP positive

            B) small number of hairy cells in peripheral blood

            C) good survival

            D) subtype of CML

            E) two of the above

35-40.  Match the following pictures to the appropriate diagnosis.  Answers may be used once, more than once, or not at all.

            A) AML

            B) ALL

            C) CML

            D) CLL

            E) multiple myeloma

            F) hairy cell leukemia

            G) Hodgkin lymphoma

            H) nodular sclerosing NHL

            I) Burkitt lymphoma

35. 

36. 

37.

38.

 

39. 

40.

 

ANSWER KEY

1.         Answer: C (see slide 21 of Neoplasia II)

2.         Answer: C (see slide 25 of Neoplasia II)

3-6       Answer: 3-C, 4-A, 5-B, C-D  (see slide 11 of Lymphomas, 8 of Leukemias)

7.         Answer: A (see slide 25-26 of Lymphomas)

8          Answer: B (see slide 27 of Lymphomas)

9-16     Answer: 9-B, 10-D, 11-C, 12-D, 13-A, 14-A, 15-C, 16-C (slide 32, 37, 38 of Lymphomas)

17.       Answer: D (see slide 35 of Lymphomas)

18.       Answer: A (slide 43 of Lymphomas)

19.       Answer: C (slide 45 of Lymphomas)

20.       Answer: B, false (slide 47 of Lymphomas)

21.       Answer: E (page 51 of Lymphomas)

22.       Answer: B, false (slide 55 of Lymphomas)

23.       Answer; I (slide 55 of Lymphomas)

24.       Answer: D (slide 58 of Lymphomas)

25.       Answer: B (slide 68 of Lymphomas)

26.       Answer: D (slide 44 of Leukemias)

27.       Answer: B Slide 15, 16, 17 of Leukemia)

28.       Answer: A, true (slide 31 of Leukemias)

29.       Answer: False (slide 40 of Leukemias)

30.       Answer: D (slide 43 of Leukemias)

31.       Answer: A, true (slide 45 of Leukemias)

32.       Answer: D (slide 46-47 of Leukemias)

33.       Answer: B (slide 50 of Lekuemias)

34.       Answer: D (slide 54, 61 of Leukemias)

35-40.  Answer: 35 – E, 36 – G, 37 – C, 38 – D, 39 – I, 40 – A

BEN'S review

BEN'S NOMENCLATURE REVIEW - as promised:)

Based on parenchymal components: benign or malignant

• Suffix –oma attached to cell of origin (i.e. chondroma, fibroma, osteoma)

A benign epithelial tumor projecting from the surrounding surface. A

           -Papilloma: benign epithelial neoplasm producing microscopic or macroscopic finger-like projections from the surface. Projections are exophytic since they show outward growth from the surface.

            -Adenoma: benign epithelial neoplasm which forms glandular patterns or is derived from glands (i.e. colonic adenoma).

Hamartoma of the breast

            -Hamartoma: benign mass of disorganized mature, specialized cells or tissue indigenous to a particular site (i.e. hamartoma of the breast)

Misnomas (malignant but don’t sound like it)	Mixed Tissue
-        lymphoma -        multiple myeloma -        melanoma -        seminoma -        mesothelioma -        hepatoma (hepatocellular carcinoma) -        hypernephroma	-        Teratoma o   Tissue from germ lines in abnormal sites o   Mature and immature o   Benign, ovaries/testes are common targets -        Hamartoma o   Tissue that’s in normal site but not organized properly o   benign

	Epithelial	Mesenchymal	Heme	Neuro
Benign	-oma Aden- Pappil-	-oma Lipo- Fibro- Osteo- Leio- Rhabdomyo- Lymphangio- Gemeangio-	?	-oma Schwannoma Astrocytoma (benign but graded)
Malignant	-carcinoma Adeno- Squamous cell- Hepatocellular- Renal cell- Transitional cell-	-sarcoma Lipo- Fibro- Osteo- Leio- Rhabdomyo- Lymphangio- hemeangio-	Leukemia lymphoma	-oma/-blastoma Retino- Neuro- Medull- Glio-

Courtesy of Ben T.

coagulopathy/blood product A

	PT	PTT	BT
Hemophilia A	-	increase	-
Factor 7 deficiency	increase	-	-
Factor 5 deficiency	increase	increase	-
vWF deficiency	-	increase	increase
Aspirin	-	-	Increase
Thrombocytopenia	-	-	increase
DIC	Increase	Increase	increase

heparin:

Warfarin: 

• what type of blood product is given for:
•  fibrinogen deficiency: cryopercipitate
• lacking multiple coagulation factors: fresh frozen plasma
• second line for vWF disease: cryopercipitate
• second line for hemophilia A: Cryopercipitate
• Hgb <7: packed RBC

• If an invasive procedure: how high do you want platelet count (>50,000)
• what if it is a neuro surgery (>100,000)

• How do I avoid/reduce the risk of the following reactions:
• acute hemolytic rxn: ABO match
• delayed hemolytic transfusion: good history of prior transfusions
• febrile non hemolytic rxn: leukocyte reduced (remove WBC)
• anaphylaxis - most common IgA def. recipient with anti-IgA AB
• TRALI - transfusion related acute lung injury: HLA match (antibody to HLA on WBC --- activated PMN accumulate in lungs --> microvascular occlusion and capillary leakage)
• circulatory overload: slow infusion, concentrate products/split units (ex: give packed RBCs not whole blood)
• septic rxn: proper storage / culture platelets
• graft vs host disease: Irradiation (prevent proliferation lymphocytes)

coagulopathy/blood products

	PT	PTT	BT
Hemophilia A
Factor 7 deficiency
Factor 5 deficiency
vWF deficiency
Aspirin
Thrombocytopenia
DIC

• what type of blood product is given for:
•  fibrinogen deficiency
• lacking multiple coagulation factors
• second line for vWF disease
• second line for hemophilia A
• Hgb <7

• If an invasive procedure: how high do you want platelet count
• what if it is a neuro surgery

• How do I avoid/reduce the risk of the following reactions:
• acute hemolytic rxn
• delayed hemolytic transfusion
• febrile non hemolytic rxn
• anaphylaxis
• TRALI - transfusion related acute lung injury
• circulatory overload
• septic rxn
• graft vs host disease

E2 Answers

E2 G6PD Deficiency

The two vignettes below describe the same disease process. Read both of them and then answer the following questions.

Clinical Vignette 1
A 23-year-old man of Iranian origin consults his primary care provider about a skin rash. He has an intensely pruritic rash over his buttocks and back that has appeared spontaneously. He is referred to a dermatologist, who diagnoses dermatitis herpetiformis and prescribes dapsone. Two days later he develops severe nausea and exhaustion, and complains of passing dark urine. His wife notices he has become jaundiced. He goes to the  ER, where investigations reveal anemia (Hb 7.5 g/dL) and abnormal biochemistry (elevated blood urea, deranged liver function, and unconjugated hyperbilirubinemia). An urgent hematology consult is obtained and a diagnosis of drug-induced hemolytic anemia made.

Clinical Vignette 2
An 18-year-old Greek man presents to the ER with severe nausea, vomiting, and diarrhea. His mother explains that he had been at a lunch party with friends and none of the other guests were ill. The patient had ingested a meal of rice, meat, and freshly cooked beans. He has not had any significant illnesses in the past. Examination reveals a markedly dehydrated young man who is clinically anemic and jaundiced. Investigations show a hemoglobin of 5.1 g/dL, elevated WBC count with a predominant neutrophilia, elevated blood urea and creatinine, and deranged liver function. No urine can be obtained. Intravenous fluids are commenced, followed by a transfusion of packed red cells; the patient becomes acutely dyspneic, however, and chest x-ray shows features of pulmonary edema. A nephrologist is consulted. Intravenous diuretic therapy is prescribed, a urinary catheter inserted, and 30 mL of urine obtained that, on testing, shows a high urobilinogen and protein content.


A peripheral smear was done and is show below for both of these patients. What abnormalities do you see if any? Which of the two images was taken during active disease?

There are two images. The top left corner shows a supravital stain with Heinz bodies, which are inclusions representing denatured hemoglobin. The peripheral smear in the main box shows bite cells. Active hemolysis would be found during active intravascular hemolysis.


What do you think caused their visit to the ER? Name a reason for each patient. Was it an environmental or genetic cause? What is the their condition? Name other inciting factors.

Exposure to Dapsone. Ingestion of fava beans.

Genetic and Environmental. G6PD is an x-linked recessive(medit-greeks/italians and African americans) d/o that is activated when an oxidizing substance is encountered.

Oxidant stresses that induces hemolysis:
-infection (most common)
-Drugs (primaquine, chloroquine, dapsone, sulfonamides, nitrofurantoin)
-Fava beans (esp in Mediterranean variant)

What is a benefit of this condition? What anemia has the same benefit as this condition?

Plasmodium falciparum. Sickle cell anemia.

What is the main pathogenesis of this disease process in 3 sentences or less?

Decreased synthesis of reduced form of nicotinamide adenine dinucleotide phosphate(NADPH) and glutathoine (GSH) in the pentose phosphate pathway.

-GSH normally neutralizes hydrogen peroxide, an oxidant product in RBC metabolism
-in G6PD deficiency, peroxide oxidizes Hb, which precipitates in the form of Heinz bodies.

What is the treatment?

Avoid exposure to known oxidants. Usually hemolysis is self-limiting, with spontaneous resolution in a week or so.

(Clinical Vignette Adapted from EPOCRATES)

E2

The two vignettes below describe the same disease process. Read both of them and then answer the following questions.

Clinical Vignette 1
A 23-year-old man of Iranian origin consults his primary care provider about a skin rash. He has an intensely pruritic rash over his buttocks and back that has appeared spontaneously. He is referred to a dermatologist, who diagnoses dermatitis herpetiformis and prescribes dapsone. Two days later he develops severe nausea and exhaustion, and complains of passing dark urine. His wife notices he has become jaundiced. He goes to the  ER, where investigations reveal anemia (Hb 7.5 g/dL) and abnormal biochemistry (elevated blood urea, deranged liver function, and unconjugated hyperbilirubinemia). An urgent hematology consult is obtained and a diagnosis of drug-induced hemolytic anemia made.

Clinical Vignette 2
An 18-year-old Greek man presents to the ER with severe nausea, vomiting, and diarrhea. His mother explains that he had been at a lunch party with friends and none of the other guests were ill. The patient had ingested a meal of rice, meat, and freshly cooked beans. He has not had any significant illnesses in the past. Examination reveals a markedly dehydrated young man who is clinically anemic and jaundiced. Investigations show a hemoglobin of 5.1 g/dL, elevated WBC count with a predominant neutrophilia, elevated blood urea and creatinine, and deranged liver function. No urine can be obtained. Intravenous fluids are commenced, followed by a transfusion of packed red cells; the patient becomes acutely dyspneic, however, and chest x-ray shows features of pulmonary edema. A nephrologist is consulted. Intravenous diuretic therapy is prescribed, a urinary catheter inserted, and 30 mL of urine obtained that, on testing, shows a high urobilinogen and protein content.

1. A peripheral smear was done and is show below for both of these patients. What abnormalities do you see if any? Which of the two images was taken during active disease?

2. What do you think caused their visit to the ER? Name a reason for each patient. Was it an environmental or genetic cause? What is the their condition? Name other inciting factors. 


3. What is a benefit of this condition? What anemia has the same benefit as this condition?

4. What is the main pathogenesis of this disease process in 3 sentences or less?

5. What is the treatment?

Ben T.
(Clinical Vignettes adapted from EPOCRATES)