Term
| What are the two main morphologies of Hodgkin Lymphoma (HL)? |
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Definition
| Nodular Lymphocyte Predominant HL (NLPHL) and Classical Hodgkin Lymphoma (CHL) |
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Term
| CHL has monoclonal germal centers with What cells ? |
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Definition
| Mononuclear Hodgkin cells and Multinucleated Reed-Sternberg (RS) cells with an infiltrate of other cells like eosinophils, neutrophils, etc |
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Term
| What happens to the Nodal architecture in CHL? |
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Definition
| Effaced with variable numbers of neoplastic cells in an inflammatory backgroud |
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Term
| What type of appearance can Reed-Sternberg cells have? |
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Definition
| Mummified forms and Owl's eyes (10-11) |
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Term
| What immunophenotype do Hodgkin Reed-Sternberg cells have? |
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Definition
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Term
| What cell are H Reed-Sternberg cells derived from? |
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Definition
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Term
| What role does the active attraction by H Reed-Sternberg cells of other immune cells have? |
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Definition
| Promote growth and inhibit an immune response by "hiding" |
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Term
| What are the 4 subtypes of Classical HL, based on characteristics of the reactive infiltrate and the morphology of the Reed Sternberg (RS) cells? |
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Definition
| 1) Nodular Sclerosis, 2) Mixed Cellularity, 3) Lymphocyte Rich, 4) Lymphocyte Depeleted |
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Term
| Since the Immunophenotype and Genetic features of the subclasses of Classical Hodgkin Lymphoma are all identical, what separates them into different subclasses? |
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Definition
| Morphology, Clinical features, and associations with EBV |
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Term
| What is the most common subtype of Classical HL by far? |
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Definition
| Nodular Sclerosis (70% of cases) |
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Term
| What characteristics give Nodular Sclerosis HL its characteristic name and what morphology do the RS cells take on and what cell infiltrate is common? |
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Definition
| Collagen fibers form within the lymph nodes along with RS cells that look like "Lacunar Cells" and Eosinophils are common |
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Term
| Classically, who is affected in Nodular Sclerosis HL (age/sex) and where are the nodules typically found? |
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Definition
| Young Female; nodules found in Neck or Mediastinum |
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Term
| What classification of HL is basically a "grab-bag" with different cell types? |
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Definition
| Mixed Cellularity HL (Reed-Sternberg cells surrounded by all sorts of other cells) |
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Term
| Mixed Cellularity HL affects what sex/age primarily? |
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Definition
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Term
| Differentiate the tissue involvement between Nodular Sclerosis HL and Mixed Cellularity HL |
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Definition
| No mediastinal involvement in Mixed Cellularity HL but it will involve Spleen and BM frequently, unlike Nodular Sclerosis HL |
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Term
| Nodular Lymphocyte Predominant HL predominantly affects what sex and what is the prognosis? |
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Definition
| Affects Males; Has excellent prognosis but recurrence is very common |
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Term
| Though similar in name, how can you differentiate Nodular Lymphocyte Predominant HL (NLPHL) to Nodular Sclerosis HL (NS HL) |
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Definition
| NS HL has lots of collagen bands and fibrosis; NLPHL has no fibrosis and is lymphocyte predominant |
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Term
| What characteristic cells are found within NLPHL? |
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Definition
| LP cells or Popcorn cells (as opposed to RS cells found in Classical Hodgkin's Lymphoma) |
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Term
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Definition
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Term
| Overall, what does prognosis of Hodgkin Lymphoma depend on? |
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Definition
| the STAGE, NOT THE HISTOLOGICAL subtype (opposite non-HL) |
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Term
| What is believed to play a role in the impaired Ig production of RS (not seen in NLPHL)? |
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Definition
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Term
| What are the "B symptoms" associated with Hodgkin's Disease? |
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Definition
| Fever, Chills, Weight loss, Night sweats |
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Term
| Immunophenotype of Plasma Cells |
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Definition
| CD38+, CD138+, Cytoplasmic Immunoglobulin heavy/light chains |
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Term
| What is the hallmark of plasma cell disorders? |
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Definition
| The presence of an overproduced immunoglobulin (M or Monoclonal spike present or broad increase) in the serum called Paraprotein |
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Term
| What are Bence-Jones proteins and what can they cause? |
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Definition
| Free light-chains in urine which can damage kidneys |
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Term
| A lack of normal diversity of Immunoglobulin production, such as in plasma cell disorders, predisposes the patient to what? |
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Definition
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Term
| What is the spectrum of Plasma Cell disorders from Normal To worst? |
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Definition
| Normal > MGUS > Indolent Myeloma > Multiple Myeloma |
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Term
| What is the typical presentation of plasma cell disorders? |
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Definition
| Increased protein in serum, CRAB: hyperCalcemia, Renal dysfunction, Anemia, Bone pain |
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Term
| MGUS is defined as what serum concentration of Immunoglobulins, how stable should it be, and are there any symptoms? |
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Definition
| <3g/dL that is Stable over time (really, it appears like Multiple Myeloma with M spike but there is no other symptoms) |
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Term
| What is the progression of MGUS and why is this bad? |
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Definition
| 1% per year progression to multiple myeloma or Waldenstrom's |
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Term
| What is Plasma Cell Myeloma (multiple myeloma)? |
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Definition
| A group of diseases that involve malignant proliferation of Ig-secreting cells of B-cell lineage that are usually associated with paraproteinemia or paraproteinuria |
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Term
| What is the Pathogenesis of Plasma Cell Myeloma? |
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Definition
| IL-6 contributes to the lack of apoptosis of plasma cells |
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Term
| What does IL-6 do to bones? |
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Definition
| Contributes to bone loss by stimulating Osteoclasts and inhibiting bone formation |
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Term
| When Myeloma cells adhere to the stroma of bone marrow, what do they release and what does this lead to? |
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Definition
| Osteoclast activating factors as well as VEGF to lead to Myeloma Bone Disease |
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Term
| What bones are typically affected in Myeloma bone disease? |
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Definition
| Axial bones (vertebra, long bones, skull) |
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Term
| How do bone lesions appear in Plasma cell Myeloma? |
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Definition
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Term
| Plasma Cell Myeloma characteristically shows what on a blood smear? |
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Definition
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Term
| Bone marrow aspirate of Myeloma (multiple) might show white inclusions within plasma cells containing what and known as what? |
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Definition
| Dutcher Bodies containing Ig |
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Term
| What is the minimum level of monoclonal plasma cells in the bone marrow to diagnose Myeloma? |
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Definition
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Term
| What 4 related tissues must be impaired to diagnose Myeloma? |
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Definition
| CRAB: hyperCalcemia, Renal insufficiency, Anemia, Bone lesions |
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Term
| What is the best predictor of Tumor Load in Myeloma? |
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Definition
| Beta-2 Microglobulin (HLA light chain) |
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Term
| Diagnostic work-up of Multiple Myeloma includes an SPEP...what is that? |
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Definition
| Serum Protein ElectroPhoresis (shows M spike if MM) |
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Term
| M spike in multiple myeloma is usually due to what Ig's? |
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Definition
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Term
| What is "Myeloma Kidney" and what causes it? |
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Definition
| Concentrated Light chains in the kidney which later precipitate as Bence-Jones proteins. It has normal glomerular function though |
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Term
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Definition
| Deposits of light chains from Myeloma in tissues. Can cause glomerular lesions that leaks protein in urine |
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Term
| Most translocations found within Myeloma involve what chromosome? |
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Definition
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Term
| What two genetic abnormalities of Myeloma have the worst prognosis? |
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Definition
| Monosomy 13 and del(13q14) |
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Term
| What can be given to prevent bone loss in Multiple Myeloma? |
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Definition
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Term
| What concentration of M-protein is seen in Smoldering (asymptomatic) Myeloma and compare to MGUS |
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Definition
| >3g/dL compared to <3g/dL in MGUS |
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Term
| What causes Extramedullary Plasmacytoma and where are they frequently seen? |
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Definition
| Isolated plasma cell tumors found in soft tissue, frequently Upper respiratory tract |
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Term
| What is Plasma Cell Leukemia and what is its prognosis? |
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Definition
| Basically Acute Leukemia with plasma cells; Horrible prognosis even treated |
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Term
| How do individuals with Amyloidosis present? |
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Definition
| Raccoon eyes and Macroglossia |
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Term
| Most Amyloidosis is due to deposition of what? |
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Definition
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Term
| How frequently does AL Amyloidosis progress to Multiple Myeloma and what is the prognosis? |
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Definition
| Rare to progress to MM, but the prognosis is still poor |
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Term
| What is the cause of Waldenstrom's Macroglobulinemia? |
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Definition
| Extensive proliferation of small B-cells that secrete a bunch of IgM |
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Term
| What does the extensive IgM secretion in Waldenstrom's Macroglobulinemia lead to? |
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Definition
| Fatigue, Anemia, Amyloidosis, Coagulation abnormalities, Excess blood viscosity |
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Term
| What inclusions are frequently found in the nucleus and cytoplasm of the small B-cells in Waldenstrom's Macroglobulinemia? |
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Definition
| Dutcher bodies and Mott cells |
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Term
| How do you acutely treat Waldenstrom's Macroglobulinemia? |
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Definition
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