Hematology and Oncology Pathology II

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jknell
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Hematology and Oncology Pathology II
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2013-04-05 11:16:10
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  1. Acute Leukemia
    • Acute Leukemia:
    • -neoplastic proliferation of blasts
    • - >20% blasts in BM
    • -crowd out normal hematopoiesis
    • -usually seen in kids and teenagers

    • Subtypes:
    • 1. Acute Lymphoblastic Leukemia (ALL)
    • -B-ALL
    • -T-ALL
    • 2. Acute Myelogenous Leukemia (AML)
    • -Acute Promyelocytic Leukemia
    • -etc
  2. Chronic Leukemia
    -background
    -subtypes
    • Chronic Leukemia:
    • -neoplasm of mature circulating lymphocytes
    • -characterized by high WBC
    • -insidious onset
    • -seen in older adults

    • Subtypes:
    • 1. Lymphoid
    • -Chronic Lymphocytic Leukemia (CLL)
    • -Small Lymphocytic Lymphoma (SLL)
    • -Hairy Cell Leukemia
    • -Adult T Cell Leukemia (ATLL) (NHL)
    • -Mycosis Fungoides (NHL)

    • 2. Myeloid
    • -Chronic Myeloid Leukemia
    • -Myeloproliferative Disorders
  3. Myeloproliferative Disorders (MPD)
    -background
    -complications
    subtypes
    • Background:
    • -neoplastic proliferation of mature myeloid cells
    • -hypercellular bone marrow
    • -increase in all cells of myeloid lineage

    • Complications:
    • -hyperuricemia and gout (turnover)
    • -marrow fibrosis
    • -transformation to acute leukemia

    • Subtypes:
    • -Chronic myeloid leukemia (CML) (Granylocytes)
    • -Polycythemia Vera (RBCs)
    • -Essential Thrombocythemia (Plts)
    • -Myelofibrosis (megakaryocytes)
  4. Lyphoma
    -background
    -subtypes
    • -neoplastic proliferation of lymphoid cells that forms a mass
    • -may arise in a LN or in extranodal tissue

    Hodgkin Lymphoma (40%)

    • Non-Hodgkin Lymphoma (60%)
    • 1. Neoplasms of Mature B Cells:
    • -Follicular lymphoma
    • -Mantle Cell Lymphoma
    • -Marginal Zone Lymphoma
    • -Burkitt Lymphoma
    • -Diffuse Large B cell Lymphoma
    • 2. Neoplasms of Mature T Cells (also Chronic Leukemia)
    • -Adult T Cell Lymphoma
    • -Mycosis Fungoides/Sezary Syndrome

  5. Leukemoid Reaction
    -features
    -vs. CML
    • Features:
    • -acute inflammatory response to infection
    • -↑ WBC count
    • -↑ neutrophils and neutrophil precursors (bands, Left shift)
    • -↑ leukocyte alkaline phosphatase (killing to infection)

    • vs CML:
    • -↓ LAP (having a neoplastic party)
    • -increased basophils
    • -t(9;22)
  6. Non-Hodgkin's vs Hodgkin's Lymphoma
  7. Hodgkin's Lymphoma
    -pathophysiology
    -types and prognosis
    • Pathophysiology:
    • -proliferation of Reed-Sternberg cells
    • -RS cells secrete cytokines that attract reactive lymphocytes and may lead to fibrosis

    • Reed Sternberg Cells
    • -binucleate or bilobed
    • -"owl's eye"
    • -CD30+, CD15+, CD20- (B cell origin)

    • Nodular Sclerosing Form:
    • -most common
    • -affects women and men equally
    • -RS cells in lake-like spaces
    • -nodules divided by bands of sclerosis
    • -best prognosis

    • Lymphocyte-Depleted Form:
    • -most aggressive form
    • -elderly and HIV positive
  8. Follicular Lymphoma
    -epidemiology
    -pathophysiology
    -presentation
    -treatment
    • Epidemiology:
    • -adults

    • Pathophysiology:
    • -t(14;18)
    • -translocation of heavy chain Ig (14) and Bcl2 (18)
    • -Bcl2 promotes apoptosis
    • -Bcl2 expressed in most cells in the body but not follicular B cells (want apoptosis during SHM)
    • -expansion of follicle

    • Presentation:
    • -painless lymphadenopathy
    • -indolent course
    • -difficult to cure

    • Treatment:
    • -low dose chemo
    • -rituximab
  9. Mantle Cell Lymphoma
    -epidemiology
    -pathophysiology
    • Epidemiology:
    • -Older males

    • Pathophysiology:
    • -t(11;14)
    • -translocation of cyclin D1 (11) and heavy chain Ig (14)
    • -cyclin D1 promotes G1/S transition
    • -expansion of mantle zone
  10. Marginal Zone Lymphoma
    • Pathophysiology:
    • -associated with chronic inflammatory state
    • -marginal zone is formed by activated cells
    • -MALToma (H pylori)
    • -expansion of marginal zone
  11. Burkitt's Lymphoma
    -epidemiology
    -pathophysiology
    -presentation
    -pathology
    • Epidemiology:
    • -adolescents
    • -young adults

    • Pathophysiology:
    • -t(8;14)
    • -translocation of c-myc (8) and Ig heavy chain (14)
    • -associated with EBV

    • Presentation:
    • -African form: jaw
    • -Sporadic Form: abdomen or pelvis

    • Pathology:
    • -"starry sky" appearance (sheets of lymphocytes with interspersed MPs)
  12. Diffuse Large B-cell Lymphoma
    -epidemiology
    -pathophysiology
    -prognosis
    • Epidemiology:
    • -usually older adults
    • -20% in children
    • -most common form of adult NHL

    • Pathophysiology:
    • -sporadic
    • -transformation from low-grade lymphoma (follicular)
    • -grow diffusely in sheets
    • -may be mature T cell in origin (20%)

    • Prognosis:
    • -clinically aggressive
  13. Multiple Myeloma
    -epidemiology
    -pathophysiology
    -findings
    -associated with
    • Epidemiology:
    • -most common primary bone tumor in elderly (>40-50)

    • Pathophysiology:
    • -malignant proliferation of plasma cells in the bone marrow
    • -monoclonal: IgG (55%) or IgA (25%)

    • Findings:
    • -M spike on protein electrophoresis
    • -high serum IL6 sometimes present
    • -Bence Jones protein in urine
    • -Rouleaux formation
    • -numerous plasma cell with "clock face" chromatin and intracytoplasmic inclusions containing Ig

    • CRAB
    • -hyperCalcemia
    • -Renal insufficiency
    • -Anemia (normochromic, normocytic)
    • -Bone lytic lesions/Back pain

    • Associated with:
    • -increased susceptibility to infections
    • -primary amyloidosis (AL)

    • Distinguish from:
    • -Waldenstrom's Macroglobulinemia
  14. Waldenstrom Macroglobulinemia
    -pathophysiology
    -findings
    • Pathophysiology:
    • -B cell lymphoma with monoclonal IgM production
    • -IgM is a pentamer → hyperviscosity

    • Findings:
    • -M spike (IgM)
    • -lymphadenopathy
    • -bone lesions are absent
    • -visual and neurologic deficits (serum hyperviscosity)
  15. MGUS
    Monoclonal Gammopathy of Undetermined Significance

    • Epidemiology:
    • -common in elderly (5% of 70 yo)

    • Pathophysiology:
    • -monoclonal expansion of plasma cells with M spike
    • -other features of multiple myeloma are absent

    • Prognosis:
    • -1% develop multiple myeloma every year
  16. Acute Lymphoblastic Leukemia/Lymphoma (ALL)
    -epidemiology
    -pathophysiology
    -B-ALL
    -T-ALL
    • Epidemiology:
    • -<15 years
    • -associated in children with Down syndrome (>5 years)

    • Pathophysiology:
    • -accumulation of lymphoblasts in bone marrow
    • -lymphoblasts are TdT+, CALLA+
    • -called lymphoma when forms a mass in the mediastinum (T-ALL)
    • -may spread to CNS and testes

    • B-ALL
    • -most common type of ALL
    • -CD10+, CD19+, CD20+
    • -t(12;21): good prognosis
    • -t(9;22) Ph+ (poor prognosis)

    • T-ALL
    • -CD2-CD8+
    • -mediastinal thymic mass (lymphoma)

  17. Chronic Lymphocytic Leukemia (CLL)/ Small Lymphocytic Lymphoma (SLL)
    -epidemiology
    -pathophysiology
    -findings
    -complications
    -SLL
    • Epidemiology:
    • -> 60 years old
    • -most common leukemia over all

    • Pathophysiology:
    • -malignant proliferation of naive B cells
    • -CD5+ (usually a T cell marker) and CD20+

    • Findings:
    • -smudge cells

    • Complications:
    • -hypogammaglobulinemia: Infection (most common cause of death)
    • -autoimmune hemolytic anemia
    • -transformation to diffuse larger B-cell lymphoma (Richter transformation)

    • SLL:
    • -same as CLL but has increased peripheral blood lymphocytosis and generalized lymphadenopathy (Lymphoma)
  18. Hairy Cell Leukemia
    • Epidemiology:
    • -adults (elderly)

    • Pathophysiology:
    • -proliferation of mature B cells

    • Findings:
    • -"hairy" cells
    • -stains TRAP positive (tartrate-resistant acid phosphatase)
    • -splenomegaly (accumulation in red pulp)
    • -dry tap on BM bx

    • Treatment:
    • -cladribine (2-CDA, adenosine analog)
  19. Adult T-Cell Leukemia/Lymphoma
    -epidemiology
    -presentation
    • Epidemiology:
    • -adults
    • -associated with HTLV-1 (Japan, West Africa and Caribbean)

    • Presentation:
    • -rash
    • -lymphadenopathy
    • -HSM
    • -lytic bone lesions with hypercalcemia
    • -aggressive

    • *can be confused with multiple myeloma
  20. Mycosis Fungoides
    -epidemiology
    -pathophysiology
    -presentation
    -sezary syndrome
    • Epidemiology:
    • -adults

    • Pathophysiology:
    • -neoplastic proliferation of mature CD4 T cells

    • Presentation:
    • -localized skin rash, plaques and nodules (Pautier microabscesses)
    • -indolent course

    • Sezary syndrome:
    • -spread to involve the blood
    • -Sezary cells (cerebriform nuclei)
  21. Acute Myelogenous Leukemia (AML)
    -epidemiology
    -pathophysiology
    -presentation
    -acute promyelocytic leukemia
    • Epidemiology:
    • -median onset 65 years

    • Pathophysiology:
    • -neoplastic accumulation of myeloblasts (>20%) in BM
    • -may also rise from myelodysplastic syndrome

    • Presentation:
    • -Auer rods (MPO aggregates)

    • Acute promyelocytic leukemia
    • -t(15;17): M3 AML subtype
    • -involves retinoic acid receptor
    • -treat with all-trans retinoic acid (ATRA) (vitamin A): causes blasts to mature
    • -treatment can release Auer rods → DIC
  22. Chronic Myelogenous Leukemia (CML)
    -epidemiology
    -pathophysiology
    -presentation
    -labs
    -complications
    -treatment
    • Epidemiology:
    • -highest incidence at 30-60 years

    • Pathophysiology:
    • -neoplastic proliferation of mature myeloid cells (especially granulocytes)
    • -t(9;12) Philadelphia chromosome
    • -myeloid stem cell proliferation

    • Presentation:
    • -increased neutrophils, metameylocytes, basophils
    • -splenomegaly
    • -very low leukocyte alkaline phosphatase (vs. leukemoid reaction)

    • Labs:
    • -↑ WBCs
    • -↑ Platelets
    • -↓ RBCs

    • Complications:
    • -may accelerate and transform to AML or ALL ("blast crisis")
    • -enlarging spleen is often followed by transformation
    • *mutation is in stem cell (can be myeloid or lymphoid)

    • Treatment:
    • -imatinib (inhibits bcr-abl tyrosine kinase)
  23. Myeloproliferative Disorders
    -basic principles
    -presentation
    -complications
    -types
    • -neoplastic proliferation of mature cells of myeloid lineage
    • -older adults

    • Common Presentation:
    • -high WBC count
    • -hypercellular bone marrow
    • -cells of ALL myeloid lineages increased but classified based on dominant cells

    • Complications:
    • -increased risk of hyperuricemia and gout (high turnover)
    • -progression to marrow fibrosis
    • -transformation to acute leukemia

    • Types:
    • -Polycythemia Vera
    • -Essential Thrombocytosis
    • -Myelofibrosis
    • -CML
  24. Polycythemia Vera
    -pathophysiology
    -presentation
    -labs
    -treatment
    • Pathophysiology:
    • -abnormal clone of HSC with constituitively active JAK2 receptors
    • -proliferation of RBCs without EPO stimulation

    • Presentation:
    • -due to hyperviscosity of blood
    • -blurry vision and HA
    • -increased risk of venous thrombosis (Budd Chiari syndrome)
    • -flushed face
    • -itching (especially after bathing)

    • Labs:
    • -↑ RBCs
    • -↑ WBCs
    • -↑ platelets


    • Treatment:
    • -phlebotomy
    • -hydroxyurea
  25. Essential Thrombocytosis
    -pathophysiology
    -presentations
    -labs
    • Pathophysiology:
    • -similar to PV but specific for megakaryocytes
    • -associated with JAK2 kinase mutation

    • Presentation:
    • -bleeding or thrombosis
    • -rarely progresses to marrow fibrosis or acute leukemia

    • Labs:
    • -↑ platelets
    • -normal RBCs (↑ in pathoma)
    • -normal WBCs (↑ in pathoma)
  26. Myelofibrosis
    • Pathophysiology:
    • -fibrotic obliteration of bone marrow
    • -excessive proliferation of megakaryocytes → ↑ PDGF
    • -PDGF causes fibrosis
    • -30-50% have JAK2 mutation

    • Presentation:
    • -splenomegaly (extramedullary hematopoiesis)
    • -tear drop cell

    "Bone marrow is crying because it's fibrosed!"
  27. t(9;22)
    • Philadelphia Chromosome
    • CML

    Philadelphia CreaML cheese
  28. t(8;14)
    • Burkitt's lymphoma
    • -C-myc activation
  29. t(11;14)
    • Mantle cell lymphoma
    • -cyclin D1 activation
  30. t(14;18)
    • Follicular lymphoma
    • -bcl2 activation
  31. t(15;17)
    M3 type of AML (responsive to ATRA)
  32. Langerhans Cell Histiocytosis
    • Pathophysiology:
    • -proliferative disorders of dendritic cells from monocyte lineage
    • -cells are functionally immature and don't stimulate T cells as APCs

    • Presentation:
    • -child with lytic bone lesions and skin rash

    • Findings:
    • -express S-100 (neural crest origin)
    • -express CD1a
    • -Birbeck granules ("tennis rackets")
  33. Polycythemias (associated diseases)
    • Relative
    • -decreased plasma volume
    • -no associated disease

    • Appropriate Absolute:
    • -increased RBC mass
    • -decreased O2 sat
    • -associated diseases: lug disease, congenital heart disease, high altitude

    • Inappropriate Absolute:
    • -increased RBC mass
    • -no change in O2 sat
    • -associated disease: RCC, Wilms tumor, cyst, HCC, hydronephrosis
    • -due to ectopic EPO

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