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4. Translocate
me up,
Sharad
My, oh,
myloid
Lymph and
let lymph
Worth 1000
words?
V, for
Vignette
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5. When this translocation is present in CML, it correlates with good prognosis, but when present in ALL correlates with relatively poor prognosis.

6. t(9; 22)

7. A patient with this translocation always has cellular features shown below, and should be treated with a specific therapy to avoid complications of DIC

8. t(15;17)

9. A child with rapidly progressing lymphocytosis and high blast count might have this favorable translocation associated with his/her blasts

10. t(12;21)

11. These two translocations might be called “the twins,” because not only are they both associated with favorable disease prognosis, they also both disturb a “twin” set of proteins needed for normal granulocyte maturation.

12. t(16;16) and t(8;21)

13. These are all three of the translocations we have learned that are associated with the IgH promoter, and the diseases they are found in.

14. t(8; 14): Burkitt’s lymphoma t(11; 14): Mantle cell lymphoma
t(14; 18): Follicular lymphoma

15. This uncommon neoplasm is probably one of the only times treatment with leeches would still work, if your patient let you try them.

16. Polycythemia vera (normally phlebotomy, not leeches)

17. This mutation underlies the pathology in no fewer than 3 myeloproliferative disorders.
Click once to show text, click again on text to start verse 1 of jeopardy song, click again for verse 2, and click again when time is up.

18. JAK2 mutation

19. In this disease, peripheral smear shows red blood cells leaving their home to in a “trail of tears”, accompanied by myelocytes and nucleated reds which fill up the spleen.

20. Primary myelofibrosis (or spent phase of other myeloproliferative disorder)

21. You might not be able to eat that whole Philly cheesesteak if you have absolute basophilia, eosinophila, and leukocytosis in the disease derived from this cellular precursor (give stage/proliferative potential).

22. Pluripotent stem cell (CML has the ability to convert to ALL or AML)

23. Erythromelalgia, a throbbing and burning of hands and feet caused by occlusion of small arterioles, is a rather characteristic symptom of this relatively indolent neoplasm – just don’t try bloodletting.

24. Essential thrombocytosis
erythromelalgia also seen in PCV, hence the bloodletting comment

25. This common neoplasm can show up almost anywhere in the body; its aggressive neoplastic cells dwarf ordinary lymphocytes.

26. Diffuse large B-cell lymphoma

27. You might be careful how crazy you get on that Caribbean cruise – you just might catch the virus that causes this rapidly fatal cancer, although you’d be safe for a good year latency period.

28. Adult T cell lymphoma

29. A person with this test result could have these three diseases, among others:

30. MGUS, multiple myeloma, Waldenstrom macroglobulinemia, primary amyloidosis, heavy chain disease (monoclonal gammopathy)

31. Your diagnosis is a ringer if you see large, ugly horseshoe-shaped nuclei in this neoplasm, which is frequently produced by a translocation involving chromosomes numbered the same as its CD-markers.

32. Anaplastic large (T) cell lymphoma
This is an uncommon disorder but it has a nice genetic basis, at least in kids, so we have to learn it: there is a t(2;5) that creates an ALK fusion protein. This fusion protein is a constitutively active tyrosine kinase that activates JAK/STAT and other growth pathways. In older adults, the translocation is more often absent.
The clinical picture is that of a soft tissue mass in pretty much any age. Histologically cells are large, anaplastic, and have horseshoe-shaped nuclei in many cases. They express T cell markers and may stain positive for ALK fusion protein. It does not completely follow the “large is bad” rule: childhood disease associated with the t(2;5) has a 75-80% cure rate, while similar-looking tumors in older adults have a worse prognosis.

33. You should be sure to keep this histopathological feature in mind when diagnosing a heterogenous group of memory B-cell neoplasms, all of which typically occur in the setting of chronic inflammation.

34. Lymphocytic infiltrate/attack of glands or other epithelial structures surrounding the MALT. (maltoma)
Extranodal marginal zone B-cell lymphoma of mucosal-associated-lymphoid-tissue (maltoma, EMZL-MALT) are lymphomas from memory B cells. Usually they occur in the setting of chronic inflammation; for example, the MALT of the stomach in H. pylori infection or the mouth in Sjörgen syndrome.

35. This CD marker is present on the neoplastic cells which give rise to this syndrome.

36. CD4 (accept other pan T-cell markers: CD2, 3, 5, 7; NOT 8)

37. This highly unique laboratory (CBC) abnormality may be the fur-st sign of the disease pictured.

38. Monocytopenia (Hairy cell leukemia)

39. This is the general age group in which you would see a patient with this peripheral smear and immunophenotype of CD2+, CD3+, CD4+, CD5+, CD7+, CD8+, CD 13-CD19-, CD20-, CD33-, CD34-, CD117-

40. Adolescents (Acute T cell leukemia)

41. The neoplastic disease pictured, which is historically very difficult to treat, is frequently mistaken for this non-neoplastic, troublesome disorder.

42. Pernicious (macrocytic) anemia (Myelodysplastic syndrome)

43. These are 3 of many signs / symptoms that may accompany this disease:

44. Roleaux formation: due to high Ig in serum. Most commonly:
- Plasma cell neoplasm (esp MM): Bone pain, lytic lesions in axial skeleton, hypercalcemia, vertebral fractures, plasma cells in bone marrow, Bence-Jones proteins in urine, serum protein electrophoresis
- Also accept features of other disease with high Ig in serum: ie malar rash (lupus), fatigue (autoimmune hemolytic anemia), etc; if justified

45. 1. Kyle comes to Dr. Kennedy because he has been losing weight, feeling fatigued, sweating at night, and running fever for over a week. He blames his symptoms on burnout from not being in clinics and seeing patients. Dr. Kennedy finds a 3 cm LN in Kyle’s armpit and biopsies it. Dr. Alderson looks at the slide and sees this:
Which of the following is true?
He has poor prognosis
His spleen is enlarged due to mets
The cancer displays a typical B-cell immunophenotype
He should have a CXR and CT to look for mets
Tyrosine kinase mutation drives this tumor’s proliferation

46. HODGKIN LYMPHOMA
He has poor prognosis - Prognosis for HL is good regardless of age group, even in advanced disease
His spleen is enlarged due to mets HL is rarely extranodal in primary presentation
The cancer displays a typical B-cell immunophenotype Though these cells are B cell derived, they have a highly atypical immunophenotype; CD15+, CD30+, sIg-, CD20+/-
He should have a CXR and CT to look for mets – reasonable since HL spreads contiguously
Tyrosine kinase mutation drives this tumor’s proliferation Unknown etiology, but EBV-associated and involves NF-kB upregulation

47. 2. Caitlin, a 63 year old woman, makes an appointment to see you about a lump on her neck. She feels fine. You find a 4 cm submandibular node, which you biopsy. Here is what it looks like:
What would be most useful to make the diagnosis?
Flow cytometry of CD19 and CD20
Translocation analysis by FISH
Bcl-2 cytochemical stain
Serum protein electrophoresis
Flow cytometry of CD11c, CD25, and CD103

48. What would be most useful to make the diagnosis?
Flow cytometry of CD19 and CD20 All this would tell you is that it is a B cell neoplasm, which is probably what it is anyway (85% of the time)
Translocation analysis by FISH Things that have small cells and grow diffusely are: CLL, mantle cell lymphoma, Burkitt lymphoma. This clinical picture most resembles either of the former, but regardless a translocation analysis would nicely stratify them: CLL – no translocation; Burkitt: t(8; 14), mantle cell: t(11; 14).
Bcl-2 cytochemical stain - follicular lymphoma does not show diffuse growth
Serum protein electrophoresis this is not a plasma cell neoplasm
Flow cytometry of CD11c, CD25, and CD103 this is characteristic of hairy cell leukemia; those aren’t hairy cells

49. 3. Jordan, a 67 year old man, goes to the hospital for a nosebleed that just won’t stop. Once he is within 10 miles of the hospital, a CBC is run on him and shows a moderate lymphocytosis and thrombocytopenia. Bone marrow biopsy shows an interstitial growth pattern of small lymphocytes, CD5+ CD23+. The lymphocytes are shown to be a clonal B-lineage proliferation with > 2% of the IgHv region differing from that of genetic libraries.
Which of the following is true concerning James’ disease?
Prognosis is poor compared to other forms
It is associated with a characteristic translocation
It is derived from a naïve B cell
It is likely to transform into a more malignant subtype
It may be associated with a paraprotein

50. Prognosis is poor compared to other forms U-CLL has worse prognosis b/c of potential for transformation
It is associated with a characteristic translocation CLL is not associated with a chromosomal translocation
It is derived from a naïve B cell – true of U-CLL
It is likely to transform into a more malignant subtype – transformation is more characteristic of U-CLL
It may be associated with a paraprotein – Hyper or hypogammaglobulinemia may be associated with CLL, resulting in immune dysfunction that can be responsible for platelet or RBC destruction and anemia and thrombocytopenia.

51. Which of the following immunophenotypes is most likely?
4. Joe is a 65 year old white male who notices that he is fatigued and bleeds for a long time when he cuts himself while shaving. This has been going on for about 6 months. His CBC reveals a moderate absolute lymphocytosis with absolute thrombocytopenia and anemia. Smudge cells and spherocytes are seen in the peripheral smear.
Which of the following immunophenotypes is most likely? a.
CD10+, CD19+, TdT+, sIg- b.
CD2+, CD3+, CD5+, CD7+, TdT+, CD4+ & CD8+ c.
CD19+, CD20+, CD5+, CD10-, sIg+, CD23+, cyclin D1- d.
CD19+, CD20+, CD5-, CD10-, sIg+, CD11c+, CD25+, CD103+ e.
CD19+, CD20+, CD10+, CD5-, sIg+, Bcl2+ f.
CD19+, CD20+, CD10+/-, CD5-, sIg+ g.
CD19-, CD20-, sIg- h.
CD19+, CD20+, CD10+, CD5-, sIg+, TdT- i.
CD19+, CD20+, CD10-, CD5+, sIg+, CD23-, cyclin D1+

52. B- ALL
B-ALL: CD10+, CD19+, TdT+, sIg-
T-ALL
CD2+, CD3+, CD5+, CD7+, TdT+, CD4+ & CD8+
CLL
CD19+, CD20+, CD5+, CD10-, sIg+, CD23+, cyclin D1-
Hairy cell
CD19+, CD20+, CD5-, CD10-, sIg+, CD11c+, CD25+, CD103+
Follicular
CD19+, CD20+, CD10+, CD5-, sIg+, Bcl2+
DLBCL*
CD19+, CD20+, CD10+/-, CD5-, sIg+
Plasma cell*
CD19-, CD20-, sIg-
Burkitt*
CD19+, CD20+, CD10+, CD5-, sIg+, TdT-
Mantle cell
CD19+, CD20+, CD10-, CD5+, sIg+, CD23-, cyclin D1+
Any T-cell-derived**
CD2+, CD3+, CD5+, TCR+ (αβ or γδ), CD4+/-, CD8+/-
Classic HL
CD15+, CD30+, CD20+/-, negative for most others
Lymph-pred. HL
CD19+, CD20+, CD15-, CD30-

53. 5. Sam, a 4 year old boy presents to your clinic with a painful lump on the top of his head which has been growing for several months. Your exam reveals exophthalmus and an overal lumpy, funny-shaped head with a 7 cm immobile, tender mass in the right upper parietal region. During the visit, he asks to leave to get a drink of water several times, and also leaves to “run pee” twice.
Which picture portrays the histopathology of the disease?

54. Hans-Christian-Schuller triad (Langerhans cell histiocytosis)
Hand-Schüller-Christian disease makes up 25% of LCH, occurs in kids from 2-5 years of age, and presents as multifocal, unisystem disease: erosive masses of the bone and surrounding soft tissue. If it affects the pituitary it can present as diabetes insipidus; exopthalmus + DI + calvarial defects is the classic HSC triad. It may spontaneously regress or go away with chemo.

55. Final Jeopardy
Make your wager

56. When you consider “the terrible two:” infection with this virus and mutation of this class of proteins, you have the etiology of the majority of leukocyte neoplasms.

57. Epstein-Barr Virus
Tyrosine kinases