diseases of white cells and lymphoid · pdf fileall/cll (acute/chronic lymphocytic leukemia)...
TRANSCRIPT
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DISEASES of WHITE CELLS and LYMPHOID TISSUE
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Topics for Chapter 14• Leukopenia/Neutropenia• Leukocytosis• Lymphadenitis/Lymphadenopathy• (Malignant) Lymphoma• NON‐Hodgkins Lymphoma• Hodgkins Lymphoma (Hodgkins Disease)• ALL/CLL (Acute/Chronic Lymphocytic Leukemia)• Multiple Myeloma• M1/M2/M3/M4/M5/M6/M7• Myeloproliferative Disorder• CML and Polycythemia Vera• Essential Thrombocytosis• Splenomegaly• Thymoma
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WBC/LYMPHOID DISORDERS
• Review of Normal WBC Structure/Function
• Benign Neutrophil and Lymphoid Disorders
• Leukemias
• Lymph Nodes
• Spleen/Thymus
• REVIEW
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NEUTROPHILS• Normal TOTAL WBC count 6‐11 K
• Neutrophils usually 2/3 of total normal
• Myeloblast Promyelocyte MyelocyteMetamyelocyte Band (stab) Mature Neutrophil (Poly, PMN, Neutrophilic Granulocyte)
• Produced in red (hematopoetic) marrow, sequester (pool) in spleen, live in peripheral blood, migrate OUT of vascular compartment PRN, live a couple days normally
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NEUTROPHIL
Neutrophil
Polymorphonuclear Leukocyte, PMN, PML
“Leukocyte”
Granulocyte, Neutrophilic granulocyte
“Poly‐”
Polymorph
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NEUTROPHIL MATURATION
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LYSOSOMAL CONSTITUENTS• PRIMARY• Also called AZUROPHILIC, or NON‐specific
• Myeloperoxidase• Lysozyme (Bact.)• Acid Hydrolases
• SECONDARY• Also called SPECIFIC
• Lactoferrin
• Lysozyme
• Alkaline Phosphatase
• Collagenase
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FUNCTIONS• Margination
• Rolling
• Adhesion
• Transmigration (Diapedesis)
• Chemotaxis
• Phagocytosis: Recognition, Engulfment, Killing (digestion)
• Equilibrium with splenic pool
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PELGER‐HUET ANOMALY• Genetic
• Sometimes ACQUIRED (Pseudo‐PELGER‐HUET)
• All neutrophils look like BANDS• NOT serious, mostly a cute incidental finding
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CHEDIAK‐HIGASHI SYNDROME• Also genetic
• Abnormal LARGE irregular neutrophil granules
• Impaired lysosomal digestion of bacteria
• Associated with pigment and bleeding disorders
• CAN be serious, especially in kids
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LEUKO‐penia/NEUTRO‐peniaNeutropenia/Agranulocytosis
• INADEQUATE PRODUCTION• INCREASED DESTRUCTION
• 500‐1000/mm3 is the DANGERzone!
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INADEQUATE PRODUCTION
• Stem cell suppression, e.g., aplastic anemias• DRUGS, esp. CHEMO, MANY antibiotics, aminopyrene, thio‐uracil, phenylbutazone
• DNA suppression due to megaloblastic/myelodysplastic states
• Kostmann Syndrome (genetic, congenital)
• Marrow usually shows granulocytic HYPO‐plasia, just as in RBC and PLAT decreased production
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INCREASED DESTRUCTION• Immune mediated
–By itself (idiopathic), or as in SLE–After “sensitization” by many drugs
• Splenic sequestration, hypersplenism• Increased peripheral demand, as in overwhelming infections, esp. fungal
• Marrow usually shows granulocytic HYPER‐plasia, just as in RBC and PLAT increased destructions
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Leukocytosis/Neutrophilia• Marrow and splenic pool size
• Rate of release between pool and circulation
• Marginating pool
• Rate of WBCs (neutrophils/monocytes) leaving the vascular compartment
• NON‐vascular pools FIFTY times larger than the vascular pools
• TNF/IL‐1/cytokines stimulate T‐cells to produce CSF, the WBC equivalent of EPO
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NEUTROPHIL INCREASES(e.g., “NEUTROPHILIA”)
• BACTERIA
• TISSUE NECROSIS, e.g., MI
• DÖHLE BODIES and TOXIC GRANULES are often seen with NEUTROPHILIA
• Accompanied by a “LEFT” shift
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EOSINOPHIL INCREASES(i.e., “EOSINOPHILIA”)
• ALLERGIES (esp. DRUG allergies)• PARASITES
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BASOPHIL INCREASES(i.e., “BASOPHILIA”)
• RARE. Period.• But if you want to remember something at least, remember myeloproliferative diseases in which ALL cell lines are increased
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MONOCYTE INCREASES(i.e., “MONOCYTOSIS”)
•TB• SBE• RICKETTSIAL DISEASES• MALARIA
• SLE• IBD, i.e., ULCERATIVE COLITIS
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LYMPHOCYTE INCREASES(i.e., “LYMPHOCYTOSIS”)
• TB• VIRAL
–Hep‐A–CMV
–EBV• Pertussis (whooping cough)
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“MYELOPROLIFERATIVE”disorders
• Also called “chronic” myeloproliferative disorders because they last for years
• ALL marrow cell lines are affected, splenomegaly
• Proliferating cells do NOT suppress residual marrow production, and go OUTSIDE marrow , and EXPAND marrow to fatty appendicular marrow
• Associated with EXTRA‐medullary hematopoesis– Chronic Myelogenous “Leukemia” (CML)
– P. Vera
– Essential Thrombasthenia (aka, Essential Thrombocytosis)
– Myelofibrosis
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CML• NOT AT ALL like an “acute” leukemia, but can develop into one as a condition called a “blast crisis”
• Age: adult, NOT kids
• 90% have the “Philadelphia” chromosome, which are aberrations on chromosome #9 (BCR) and #22 (ABL), the BCR‐ABL “fusion”
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CML• Marrow 100% cellular, NOT 50%• ALL cell lines increased, M:E ratio massively increased, 50K‐100K neutrophils with SIGNIFICANT “left shift”, but not more than 10% blasts
• SIGNIFICANT SPLENOMEGALY!!!!!• Significant breakthrough with BCR‐ABL kinaseinhibitors!!! (90% remissions)
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Polycythemia Vera• All cell lines increased, NOT just RBC
• HIGH marrow cell turnover stimulates increased purines which often cause gout (10%)
• BOTH thrombosis AND bleeding risks are present because the increased platelets are AB‐normal
• Do not get “blast” crises, BUT can progress to myelofibrosis
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ESSENTIAL THROMOCYTOSIS
• Platelet count often near 1 million/mm3
• Often a diagnosis of exclusion.
• The RAREST of all myeloproliferativedisorders
• Giant platelets usually. Why? Ans: Quicker release from marrow (RPW/RDW)
• Massively increased megakaryocytes in the marrow
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PRIMARY MYELOFIBROSIS
• Rapid progressive marrow fibrosis
• Oldest age group of all the MPD’s, >60
• Can follow other MPD’s. Why?
• Usually the most extensive extramedullary hematopoesis because the marrow is NOT the primary site of hematopoesis
• LEUKOERYTHROBLASTOSIS
• Like CML, 10‐20% can progress to AML
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WBC/LYMPHOID DISORDERS
• Review of Normal WBC Structure/Function
• Benign Neutrophil and Lymphoid Disorders
•Leukemias• Lymph Nodes
• Spleen/Thymus
• REVIEW
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LEUKEMIAS• MALIGNANT PROLIFERATIONS of WHITE BLOOD CALLS
• In the case of neutrophilic precursors, the primary process is marrow and peripheral blood, but can involve any organ or tissue which receives blood
• In the case of lymphocytes, there is an intimate concurrence with malignant lymphomas
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Leukemias vs. Lymphomas• All leukemias of lymphocytes have lymphoma counterparts
• Primary lymphomas can have “leukemic” phases, including multiple myelomas
• Any myeloid leukemia can infiltrate a lymph node, or any other site, but if/when it does it is NOT called a lymphoma, but simply a myeloid infiltrate INTO a lymph node
• ALL lymphomas are malignant proliferations of lymphocytes
• ALL leukemias involve bone marrow changes
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LYMPHOMAS• NODAL or EXTRANODAL
• T or B
• SMALL or LARGE CELLS
• FOLLICULAR or DIFFUSE
• Hodgkins or NON‐Hodgkins
• “F.A.B. classification” is currently popular
this week (FrenchAmericaBritish), for the NON‐Hodgkins lymphomas
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LEUKEMIAS• Acute or Chronic
• Myeloid or Lymphocytic
• Childhood or Adult
• All involve marrow
• All ACUTE leukemias suppress normal hematopoesis, i.e., have anemia, thrombocytopenia
• Most have chromosomal aberrations
• Some can respond DRASTICALLY to chemo, most notably ALL in children, even be cured!!!!
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WHITE CELL NEOPLASMS Leuk/Lymph
• Many have chromosomal translocations
• Can arise in inherited and/or genetic diseases:– Downs Syndrome (Trisomy 21)
– Fanconi’s anemia (hereditary aplastic anemia)
– Ataxia telangiectasia
• May have a STRONG viral relationship:– HTLV‐1 (lymphoid tumors)
– EBV (Burkitt Lymphoma)
– Human Herpesvirus‐8 (B‐Cell Lymphomas)
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WHITE CELL NEOPLASMS Leuk/Lymph
• Can be caused by H. Pylori (gastric B‐Cell lymphomas)
• Can follow celiac disease (gluten sensitive enteropathy T‐Cell lymphomas)
• Are common in HIV, T‐Cell lymphomas, CNS lymphomas
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A.L.L./LYMPHOMAS*• SUDDEN ONSET
• ANEMIA, BLEEDING, FEVER
• Bone pain, adenopathy, hepatosplenomegaly
• CNS: headaches, vomiting, nerve palsies
• (* NB: These are pretty much the symptoms of A.M.L. too and vice versa)
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A.L.L./LYMPHOMAS• “Lymphoblasts” which can give rise either to T or B cells are the cells of malignant proliferation
• All lymphocytic leukemias CANNOT be classified independently of lymphomas because they all have lymphoma counterparts
• A.L.L. mostly in children
• Most have chromosomal changes, hyperploidy, Philadelphia chromosome, translocations
• SIGNIFICANT response to chemo: 90% remission, 75% CURE!!!
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A.L.L.
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C.L.L.• Unexplained sustained (months) lymph count of > 4000/mm3 is CLL, usually picked up on CBC
• M>F
• Lymphs look normal and are NOT blasts
• No need for marrow exam for dx, but progressive involvement of marrow, nodes, and other organs is the usual biologic behavior
• Liver can be involved portally or sinusoidally
• Translocations RARE, but trisomies and deletions common
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C.L.L.
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C.L.L.• HYPO‐gammaglobulinemia
• 15% have antibodies against RBC’s or PLATS
• CANNOT be classified as separate from lymphomas
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MULTIPLE MYELOMA• DEFINED AS A MALIGNANT PROLIFERATION OF PLASMA CELLS
• Can have a “leukemic” phase, but the BONE MARROW is the usual primary site of origin
• Usually have MONOCLONAL GAMMOPATHIES
• Secrete Heavy and Light chains, and Light chains in the urine is known as Bence‐Jones protein
• Usually have elevated IL‐6 (bad prognosis)
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PLASMA CELL classic features• OVAL cytoplasm, ROUND nucleus off to side
• Cartwheel/Clockface chromatin
• Prominent Golgi or “Hoff”
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MONOCLONAL “SPIKE” on SPE
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MULTIPLE MYELOMA• BONE DESTRUCTION
• Various deletions and translocations
• Plasma cells usually 1‐3% of marrow, but >20% or plasma cells in SHEETS is diagnostic
• Plasma cells usually look normal
• IgG >> IgA, other immunoglobulins are rare
• Staph, Strep, E. coli infections
• Bleeding
• Amyloidosis
• RENAL FAILURE
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Multiple Myeloma: Skull X‐ray
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“Solitary” Plasmacytoma• Progression to MM is “inevitable”, with time, perhaps 10‐20 years even
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M.G.U.S.•Monoclonal Gammopathy of Unknown
Significance, i.e., no plasma cell proliferation is found
• Age related
• 1% of 50‐year olds, 3% of 70‐year olds, etc.
• Same chromosomal aberrations as MM, but generally follow a BENIGN course
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Other “GAMMOPATHIES”• Waldenstrom’s MACROglobulinemia (associated with lymphomas)
• Heavy Chain Disease (associated with lymphomas)
•AMYLOID, follows MM and/or chronic granulomatous diseases
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• GENETIC ABERRATIONS INHIBIT DIFFERENTIATION
• Many have various TRANSLOCATIONS
• F.A.B. classifies them as M0 M7
• MORE than 20% of BLASTS are needed in the marrow for a diagnosis of acute leukemia!!! (i.e., ANY kind of BLAST
• NORMALLY, a marrow should have only about 1‐2 % blasts
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A.M.L.• M0 Minimally differentiated
• M1 AUER rods rare (COMMON)
• M2 AUER rods common (COMMON)• M3 Acute PRO‐myelocytic leukemia
• M4 AMML (myelo‐Mono cytic) (COMMON)• M5 Monocytic
• M6 ErythroLeukemia
• M7 Acute Megakaryocytic leukemia
NOTE: Diagnosis is CONFIRMED by special markers, not just visual identification
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M0 M2
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M3
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M4‐M5
AMML
Normal “classic” monocyte
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M6‐M7
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• Anemia
• Thrombocytopenia (bleeding)– Petechiae
– Ecchymoses
• Fever
• Fatigue
• Lymphadenopathy
• 60% respond, BUT only 20 % are free of remission after 5 years, WORSE than A.L.L.
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MYELO‐DYSPLASTIC SYNDROMES• Increased risk of acute leukemias
• But, UNLIKE the myeloPROLIFERATIVE syndromes, NOT a hypercellular marrow
• Spontaneous or drug related (even > 5 yrs!)
• Has marrow ABERRATIONS
– REFRACTORY ANEMIAS
– RINGED SIDEROBLASTS (Fe in mitochondria)
– Nuclear “BUDDING”
– EXCESS BLASTS, but LESS than 20%
– About, say 25% develop into acute leukemias
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Ring Sideroblasts and “BUDS”
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LYMPH NODES• Normal Structure, Function
• Benign enlargement/Benign disease– Acute
– Chronic (follicular vs. “sinus histiocytosis”)
• Lymphomas/Malignant Lymphomas– Adjectives of various classifications
– Features
– STAGING
• Metastatic disease TO lymph nodes
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CORTEX---SUB-capsular Sinus---Follicles (Pri? Or second.?)---PARA-follicular zoneMEDULLA
Blood flow?Lymph flow?
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Definition of TERMS• Lymphadenopathy
• Lymphadenitis
• Dermatopathic
• Normal size?
• Palpation
• What to do if a lymph node is enlarged?
• Diffuse/Follicular
• T/B/NK, Small/Large, Cleaved/Non‐cleaved
• Precursor/Peripheral
• HD/Non‐HD
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BENIGN ENLARGEMENT• Also called LYMPHADENITIS, and HYPERPLASIA
• Can be ACUTE (tender), or CHRONIC (non‐tender)
• Usually SUBSIDE in, say, less than 6 weeks
• FOLLICULAR HYPERPLASIA is enlargement of the cortical secondary follicles and increase in number of the cortical secondary follicles
• SINUS HISTIOCYTOSIS is prominence in medullary sinuses (also called “reticular” hyperplasia)
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(MALIGNANT) LYMPHOMAS
• Terms in historic classifications:– Diffuse/Follicular, Small/Large, Cleaved/Non‐cleaved
– Hodgkins (REED‐STERNBERG CELL) /NON‐Hodgkins
– Lukes, Rappaport, etc.
– Working Formulation, WHO, NIH, FAB, Intl., etc.
–B–T–PRECURSOR (less mature looking)
–PERIPHERAL (more mature looking)
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DIFFUSE LYMPHOMA
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FOLLICULAR LYMPHOMA
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LARGE CELL LYMPHOMA
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SMALL CELL LYMPHOMA
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“CLEAVED” CELL LYMPHOMA
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“Hairy” Lymphocyte
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FEATURES of LYMPHOMAS• The Antigen receptor genes re‐arrangement PRECEDES malignant transformation, so the cells are MONOCLONAL, NOT the usual POLYCLONAL
• 85% B‐cell, 15% T‐Cell
• The tumor cells congregate wherever T and B cell congregate normally however
• DISRUPTED or “EFFACED” normal architecture, obliterated subcapsular sinus
• HD/Non‐HD staging CRUCIALLY IMPORTANT, esp. HD. Why? HD grows more “linearly”
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LATEST CLASSIFICATION• NON‐HODGKIN
–PRECURSOR B–PERIPHERAL B–PRECURSOR T–PERIPHERAL T
• HODGKIN’S DISEASE (i.e., HODGKINS LYMPHOMA)
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PRECURSOR B• Precursor B LYMPHOBLASTIC LEUKEMIA/LYMPHOMA
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PERIPHERAL B• CHRONIC LYMPHOCYTIC LEUKEMIA/LYMPHOMA
• B‐Cell PRO‐lymphocytic LEUKEMIA
• Lymphoplasmacytic
• Splenic and Nodal Marginal Zone
• EXTRA‐nodal Marginal Zone
• Mantle Cell
• Follicular
• Marginal Zone
• Hairy Cell Leukemia
• Plasmacytoma/Multiple Myeloma
• Diffuse B Cell
• BURKITT LYMPHOMA (Starry Sky)
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PRECURSOR T• Precursor T LYMPHOBLASTIC LEUKEMIA/LYMPHOMA
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PERIPHERAL T and NK• T‐Cell PRO‐Lymphocytic Leukemia
• Large Granular
• Mycossis fungoides/Sezary Cell syndrome (skin)
• Peripheral T‐Cell
• Anaplastic large cell
• Angioimmunoblastic T‐Cell
• Enteropathy‐associated T‐Cell
• Panniculitis‐like
• Hepatosplenic gamma‐delta
• Adult T‐Cell
• NK/T Cell nasal
• NK‐Cell leukemia
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LYMPHOCYTE MARKERS (CD‐)i.e., LYMPHOCYTE ANTIGENS
• T‐Cell: 1,3,4,5,8• B‐Cell: 10 (CALLA), 19,20,21,23,79a• Mono/Mac: 11c, 13, 14, 15, 33, 34
• STEM: 34
• RS: 15, 30• All: 45 (Leukocyte Common Antigen)
• NK: (16, 56)
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HODGKINS DISEASE• NEED R‐S (Reed‐Sternberg, or Sternberg‐Reed) cells for correct diagnosis
–NODULAR SCLEROSIS (Young Women), the R‐S cells may be called “LACUNAR” cells
–MIXED CELLULARITY– Lymphocyte RICH
– Lymphocyte POOR
– Lymphocyte PREDOMINANCE
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STERNBERG‐REED CELL
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STAGING, HD & NHD• I ONE NODE or NODE GROUP
• II MORE than ONE, but on ONE side of diaph.
• III BOTH sides of diaph., but still in nodes only
• IV OUTSIDE of NODES, e.g., liver, marrow, etc.
• A No systemic symptoms
• B fever and/or night sweats and/or 10% weight loss
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METASTATIC CARCINOMA
• Perhaps the single most important staging and prognostic feature of tumors
• The metastatic cells FIRST enter into the SUBCAPSULAR SINUS
• The tumor may replace the entire node and enlarge it
• The tumor may be focal
• The tumor usually looks the same as it’s primary or other metastases
• The tumor usually ENLARGES the node
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METASTATIC SQUAMOUS CELL CARCINOMA
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METASTATIC ADENOCARCINOMA
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SUBCAPSULAR SINUS
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SPLEEN• 150 grams POST‐LUQ (just like kidney, 1/10 of liver)
• Bordered by diaphragm, kidney, pancreas, splenic flexure, stomach
• SMOOTH & GLISTENING capsule
• 50% RED pulp, 50% WHITE pulp
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ABNORMAL SPLEEN
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ABNORMAL SPLEEN
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SPLENIC FUNCTION• REMOVE OLD BLOOD CELLS
• MAJOR SECONDARY ORGAN of the IMMUNE SYSTEM
• HEMATOPOIESIS
• SEQUESTER (POOL) BLOOD CELLS
• 15% of body’s PHAGOCYTIC activity is in the spleen (liver has >80)
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SPLENOMEGALY• CONGESTIVE vs INFILTRATIVE• HYPERSPLENISM
–Anemia
–Leukopenia–Thrombocytopenia
• DECISION for SPLENECTOMY
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SPLENOMEGALY• INFECTIONS: TB, Mono, Malaria, Fungus
• PORTAL HTN: CHF, CIRRHOSIS, PV Thromb.
• LYMPHOHEMATOGENOUS: Leuk, Lymph
• IMMUNE: RA, SLE
• STORAGE: Gaucher, Niemann‐Pick
• MISC: Amyloid, mets (melanoma, lymphoma, Germ cell tumors of testis)
LONG STANDING CONGESTION breeds FIBROSIS
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INFARCT
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PRIMARY TUMORS (RARE)
•HEMANGIOMA
• LYMPHANGIOMA• fibroma
• osteoma
• Chondroma
•LYPHOMA
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MISC• Congenital Absence (very rare)• “Accessory” spleens (very common)
•RUPTURE
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THYMUS• Mother of all T‐Cells
• Massive in newborns, virtually absent in the elderly, bilobed
• Under manubrium
• 1) Thymocytes
• 2) Epithelial Ret. Cells
• 3) Hassal’s Corpuscles
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HASSAL’s CORPUSCLES
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DISEASES•HYPOPLASIA/APLASIA
» DiGeorge Syndrome
•CYSTS (incidental)•THYMOMAS
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THYMOMAS• ALL (most) thymomas show counterparts of BOTH lymphoid as well as epithelial reticular cells, hence, the classic name “LYMPHOEPITHELIOMA”–Benign thymoma: (encapsulated)
–Malignant Thymoma I: (locally invasive)
–Malignant Thymoma II: (easily metastasizable)
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THYMOMAS