Presentation on theme: "Instructions for using this template."— Presentation transcript:
1Instructions for using this template. Remember this is Jeopardy, so where I have written “Answer” this is the prompt the students will see, and where I have “Question” should be the student’s response.To enter your questions and answers, click once on the text on the slide, then highlight and just type over what’s there to replace it. If you hit Delete or Backspace, it sometimes makes the text box disappear.When clicking on the slide to move to the next appropriate slide, be sure you see the hand, not the arrow. (If you put your cursor over a text box, it will be an arrow and WILL NOT take you to the right location.)
2You will be given the answer. You must give the correct question. JeopardyChoose a category.You will be given the answer.You must give the correct question.Click to begin.
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11These 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.
15This uncommon neoplasm is probably one of the only times treatment with leeches would still work, if your patient let you try them.
16Polycythemia vera (normally phlebotomy, not leeches)
17This 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.
19In 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.
20Primary myelofibrosis (or spent phase of other myeloproliferative disorder)
21You 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).
22Pluripotent stem cell (CML has the ability to convert to ALL or AML)
23Erythromelalgia, 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.
24Essential thrombocytosis erythromelalgia also seen in PCV, hence the bloodletting comment
25This common neoplasm can show up almost anywhere in the body; its aggressive neoplastic cells dwarf ordinary lymphocytes.
31Your 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.
32Anaplastic 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.
33You 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.
34Lymphocytic 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.
35This CD marker is present on the neoplastic cells which give rise to this syndrome.
36CD4 (accept other pan T-cell markers: CD2, 3, 5, 7; NOT 8)
37This highly unique laboratory (CBC) abnormality may be the fur-st sign of the disease pictured.
43These are 3 of many signs / symptoms that may accompany this disease:
44Roleaux 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
451. 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 prognosisHis spleen is enlarged due to metsThe cancer displays a typical B-cell immunophenotypeHe should have a CXR and CT to look for metsTyrosine kinase mutation drives this tumor’s proliferation
46HODGKIN LYMPHOMAHe has poor prognosis - Prognosis for HL is good regardless of age group, even in advanced diseaseHis spleen is enlarged due to mets HL is rarely extranodal in primary presentationThe 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 contiguouslyTyrosine kinase mutation drives this tumor’s proliferation Unknown etiology, but EBV-associated and involves NF-kB upregulation
472. 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 CD20Translocation analysis by FISHBcl-2 cytochemical stainSerum protein electrophoresisFlow cytometry of CD11c, CD25, and CD103
48What 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 growthSerum protein electrophoresis this is not a plasma cell neoplasmFlow cytometry of CD11c, CD25, and CD103 this is characteristic of hairy cell leukemia; those aren’t hairy cells
493. 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 formsIt is associated with a characteristic translocationIt is derived from a naïve B cellIt is likely to transform into a more malignant subtypeIt may be associated with a paraprotein
50Prognosis is poor compared to other forms U-CLL has worse prognosis b/c of potential for transformationIt is associated with a characteristic translocation CLL is not associated with a chromosomal translocationIt is derived from a naïve B cell – true of U-CLLIt is likely to transform into a more malignant subtype – transformation is more characteristic of U-CLLIt 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.
51Which 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+
535. 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?
54Hans-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.