Published on

1 Comment
No Downloads
Total views
On SlideShare
From Embeds
Number of Embeds
Embeds 0
No embeds

No notes for slide
  • Many names, same cell
  • ALL blasts look the same on routine stains, whether they are myeloblasts, lymphoblasts, monoblasts, etc.
  • TOXIC GRANULES are EXAGGERATIONS of the marrow’s normal granularity, DOHLE bodies are fragments of remaining dilated rough ER
  • Not only are basophils RARE to find normally, but pure “basophilia” is also VERY rare.
  • Why would monocytosis be linked to granulomatous diseases? Answer: Monocytes are macrophages in circulation, and granulomatous diseaseas are macrophage diseases.
  • EXTRAMEDULLARY HEMATOPOESIS is most common in the spleen, liver, and lymph nodes.
  • If a CML had much more than 10% blasts, you might suspect that the patient was going into a “blast crisis”.
  • This marrow is virtually 100% cellularity!!! This is the HALLMARK of CML, and all the cells are still marrow cells although blasts are INCREASED, i.e., more than 1-2 % This marrow is virtually 100% cellularity!!! This is the HALLMARK of CML, and all the cells are still marrow cells although blasts are INCREASED, i.e., more than 1-2 %
  • This marrow is virtually 100% cellularity!!! This is the HALLMARK of CML, and all the cells are still marrow cells although blasts are INCREASED, i.e., more than 1-2 %. In this CML megakaryocytes are proliferating so what OTHER myeloproliferative disease could this be confused with? Ans: essential thrombocythemia (essential thrombocytosis)
  • Note most of the marrow looks “fibrotic”. What stain could help you confirm that this is fibrous tissue? (trichrome)
  • The most life saving thing you can learn today is how to recognize a blast! HUGE NUCLEUS NUCLEOLI (stain LIGHTER not DARKER than the rest of the nucleus on Wright stain), How many nucleoli does that one blast cell have? Answer: 3 NO cytoplasmic differentiation
  • Many cells from CLL have a “smudge” or “basket” appearance
  • Please those THREE diagnostic features of plasma cells, the malignant plasma cells of MM look like normal plasma cells usually.
  • Normal on left, myeloma on right.
  • Note the “lytic” lesions
  • Blasts, blasts with AUER rods
  • Acute promyelocytic leukemia, remember promyelocytes have BOTH nucleoli AND nonspecific granules, true BLASTS do NOT have granules.
  • In AMML, M4, many of the peripheral leukemic cells look like monocytes, while in M5, Acute Monocytic Leukemia, MOST of them look like monocytes
  • In M6, many of the cells may resemble erythroid cells, in M7, many of the cells may resemble megakaryocytes
  • Know the difference between a myelo-”proliferative” and a myelo-”dysplastic” disease.
  • BENIGN FOLLICULAR HYPERPLASIA. Larger and more numerous than normal follicles. MEDULLA may be compromised.
  • BENIGN SINUS HISTIOCYTOSIS. The cortical area may be compromised. SINUS HISTIOCYTOSIS may be seen in reaction to cancer, even if there are NO tumor cells in the lymph node.
  • “ HAIRY” cell leukemia/lymphoma consists of lymphocytes which look hairy.
  • Most pathologists HATE lymphoma classifications with a passion!
  • I HATE this slide.
  • I HATE this slide even worse.
  • Prognosis of HD disease is related directly of percentage of lymphocytes and inversely to number of RS cells.
  • STERNBERG REED cells are called “lacunar” cells in one of the most common forms of HD called NODULAR SCLEROSING
  • The subcapsular sinus of the lymph node is the FIRST place you will spot a metastatic tumor nest!
  • Notice the “confluence” of WHITE pulp? Could this be lymphoma involvement? Ans: Yes Could this be granulomas? Ans: YES
  • Portal hypertension, prominence of RED pulp
  • Note all these are benign. The commonest MALIGNANT tumor primary to the spleen is a LYMPHOMA
  • Hassal’s corpuscles are fused epithelial reticular cells
  • Wbc

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