Ch 20: Lymphoid Disorders 2 Flashcards
What is LPL (Lymphoplastic lymphoma) similar to? (Difference?)
MALT (different site)
What kind of cells are involved in LPL? Pattern?
Mature B cell neoplasm, small lymphocytes, plasmacytoid lymphocytes, and plasma cells. Diffuse pattern
Where is LPL found?
Bone marrow. Sometimes spleen/lymph nodes
LPL can develop into? Associated with?
Waldenstrom macroglobulinemia. Hyper-viscosity syndrome
Who gets LPL?
Mostly men, 50s-60s
What nuclear pseudoinclusions are often found in LPL?
Dutcher bodies (immunoglobulin)
What LPL marker is usually found in the serum
IgM
What is the prognosis for LPL?
5-10 years
What kind of cells are involved in HCL (Hairy Cell Leukemia)? Where? What breakfast item are they compared to?
Clonal B-cells small/medium pale cytoplasm, hair-like cell cytoplasmic protrusions. Bone marrow and blood Fried eggs
Who gets HCL? Common involvement? Not?
Old men 5:1. Liver/splee. Not lymph nodes
HCL cells are positive for what marker?
TRAP (Tartrate-resistant acid phosphatase)
Drug used for remission of HCL?
2-CDA
Plasma cell neoplasias produce what kind of cells?
Terminally differentiated plasma cells capable of monoclonal gammopathy
What 5 disorders make up plasma cell neoplasias. Most important being which two?
1) MGUS 2) Multiple Myeloma 3) Plasmacytoma 4) (Ig deposition) Amyloidosis/light-chain disease 5) Osteosclerotic Myeloma (POEMS) MGUS and Multiple Myeloma
3 risk factors for Plasma Cell Neoplasia?
1) Genetics 2) Ionizing radiation 3) Chronic antigenics
Monoclonal Gammopathy of Undetermined Significance (MGUS) has what main marker?
elevated M spike in serum – IgM
What is the most common primary bone cancer?
Multiple Myeloma aka Plasma Cell Myeloma.
What defines Multiple Myeloma? (4)
Plasma cells in the marrow. Extra Ig in the blood/urine (M spike). Elevated Calcium. Sometimes elevated IL-6
What 2 Ig can be found in excess with Multiple Myeloma?
IgG and IgA
Who mostly gets Mult. Myeloma? With first degree relative?
Old (black x2) men. 4X risk.
Why does MM elevate Ca?
Increased osteoclast activation factor → lytic bone lesions (RANK receptor)
What bone findings with MM?
Lytic lesions in mostly spine/skull (bone pain) → fractures
What is the key finding in MM blood smears? Why?
Rouleax. Charge is lost on RBCs causing clumping “stack of coins”.
What 3 things do MM plasma cells overproduce?
Osteoclast activating factor, Ig, and Light chain.
What is the most common morbitity with MM? Why?
Infection. Monoclonal plasma cells → no variety of antigen protection
What does extra light chain production by MM cause, leading to? Where is it found?
Light chain disease (monoclonal kappa or lambda) → Amyloidosis – blood, urine, kidney.
MM light chain excess shows up what 2 ways?
Urine (Bence-Jones proteins) and Kidney (renal failure-myeloma kidney)
What 2 normal B-Cell CD markers do MM cells lack?
CD19/20
5 common IgH translocation Oncogenes involved in MM?
cyclin D1 and D3, C-MAF, FGFR3/MMSET, and MAFB
Symptomatic Myeloma is characterized by what acronym of symptoms?
CRAB (hyperCalcemia, Renal insuff., Anemia, Bone lesions)
Important negative prognostic indicators for MM? (5)
t(4;14), t(14;16), t(14;20), del 17p/TP53, and increased B2 microglobulin
Where in the world are T Cell lymphomas common? What pathogens are risk factors?
Asia. HTLV-1 (ATLL) and EBV
T cell lymphomas attract what 2 cells via cytokines?
Eosinophils and benign M0s
Mature (CD4) T Cell lymphomas have what markers?
CD2-8
What type of T cells are found in peripheral lymphomas?
Alpha-beta T cells
What are 2 main symptoms found with ATLL Why? Also?
Rash and hypercalcemia. CD4 goes to the skin and lytic bone lesions. Lymphadenopathy and splenomegaly
What are the 4 groupings of T Cell lymphoma? More or less aggressive than B Cell?
Leukemic, nodal, extranodal, and cutaneous. More aggressive.
How is HTLV transmitted? (2)
Breast milk, blood exposure.
Most common form of primary (CTCL) Cutaneous T-Cell Lymphoma? What kind of cells?
Mycosis Fungoides. Mature CD4
What 2 locations is CDCL infiltration characterized by? What helps ID the latter?
Skin (pautrier microacsesses) and blood (Sezary cells). Cerebriform nuclei.
3 Stages of CDCL?
Premycotic/eczematous, plaque, tumor,
ALCL (Anaplastic Large Cell Lymphoma) Involves? Interesting fact about who it afflicts?
Lymph nodes, skin. Bimodal age distribution
Diagnostic (hallmark) cells of ALCL have what 3 components?
CD30, abundant eosinophilic cytoplasm, irregularly shaped nuclei
What virus is found in T cells of most (AILT/AITL) Angioimmunoblastic T-Cell Lymphoma? Where?
EBV. In the B cells
Main 3 features of Classical Hodgkin’s lymphoma? How does it arise?
No mass, B Cells, and Reed-Sternberg cells. Arises in a young person in a single lymph node or chain, spreading contiguously (nontender)
2 Telltale features of Reed-Sternberg cell? But?
CD30/15 (not CD20) and Multi-lobed “Owl eyes” nuclei. Can be hard to find – only 1-3% of cells in affected tissue.
Reed-Sternberg cells secreting cytokines does what? Creates?
Creates B symptoms and attracts reactive inflamm. Cells – lymphocytes/plasma cell/M0s/eosinophils → fibrosis
What makes up the bulk of Hodgkin’s tumors?
Mixed reactive inflammatory cells.
What history predisposes one to Hodgkin’s?
Mono from EBV
70% of classic Hodgkin’s is what kind? Found where?
Nodular Sclerosis. Ladies’ Neck/mediastinal Lymph Nodes
Nodular sclerosis has what feature? Where are the Reed-Sternberg cells found?
Fibrosis bands in LNs. Lacunar lakes – open areas.
Hodgkin’s has the best prognosis when it is? Lacks what 3 features?
Lymphocyte rich. Eosinophils/neutrophils/sclerosis.
Most frequent subtype of Classic Hodgkin’s with HIV? What kind of cell abounds with attraction by R-S Cells using IL-5?
MCHL (Mixed Cellularity). Eosinophils
What cells are found with (NLPHL) Nodular Lymphocyte-Predominant HL? Prognosis?
L&H “popcorn cells”. Excellent
Where does NLPHL originate?
Germinal centers, B Cells
Lymphoproliferative disorders are associated with?
Immune deficiency
Bad HLA matching techniques can lead to what kind of disorder? What population is it more common in? Why?
Posttransplant Lymphoproliferative. (PTLD) Kids. EBV
4 varieties of Posttransplant Lymphoproliferative Disorder (PTLD)?
1) Early lesions – plasmacytic hyperplasia 2) Polymorphic – Heterogenous cells (most common) 3) Monomorphic – Monoclonal B cells 4) Classic HL-type – rare, in renal transplants
Another Lymphoproliferative disorder similar to PTLD? From?
Iatrogenic Immunodeficiency-Associated LD. Immunosuppressive drugs for autoimmune disease.
Histiocytic proliferations may be both…?
Neoplastic and non.
What/how do hemophagocytic disorders cause dysregulation?
An immunologic defect results in elevation of cytokines dysregulating T cell and M0 activation.
Histiocytosis has what 4 key features?
Elevated langerhans cells, CD1a, S100, Birbeck granules (tennis racket shaped)
Reminder of what Langerhans cells are/do?
Dendritic cells in the skin/etc (from bone monocytes) that are APCs to T Cells
3 subtypes of Langerhans Cell Histiocytosis? Named disease means (pathoma)?
Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe Disease. Malignant skin
Eosinophilic granuloma 2 key pathoma things? Biopsy shows?
Unnamed, so benign and not in skin (bone). Fractures in adolescents. Langerhans/Inflamm. Cells/Eosinophils
Hand-Schuller-Christian disease has what 5 pathoma facts?
3 names = malignancy affects kids >3, scalp rash, lytic skull, diabetes Insip., expthalmos
Letterer-Siwe Disease has what 4 pathoma facts?
2 names = malignancy affects kids <2, skin rash, cystic skeletal defects, rapidly fatal.
Thymoma affects thymus epithelial cells – It’s associated with what disease? Describe the cells.
Myasthenia Gravis. Cells are plump rather than spindle shaped.
Waldenstrom Macroglobulinemia is what kind of disease?
B cell lymphoma with monoclonal IgM (BIG)
What 5 symptoms should you look for in Wald. Macro.? What shouldn’t you see?
1) Lymphadenopathy 2) IgM spike in serum 3) bad vision 4) neuropathy 5) bleeds (bad platelet aggragation). Lytic lesions
