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Heme/Onc Pathoma > Chapter 6: WBC Disorders COPY > Flashcards

Chapter 6: WBC Disorders COPY Flashcards

1
Q

leukopenia

  • neutropenia
  • lymphopenia
A

NEUTROPENIA: decr number of circulating neutrophils

causes

  • drug toxicity: damage to stem cells → decr production of WBC
  • severe infection: incr movement of neutrophils into tissues → decr in circulating amt

tx: GM-CSF or F-CSF to boost granulocyte production

LYMPHOPENIA: decr number of circulating lymphocytes

causes

  • immunodef
  • high cortisol state: induced apop of lymphocytes
  • autoimmune destruction
  • whole body radiation
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2
Q

leukocytosis

A

incr circulating neutrophils

causes:

  • bacterial infection or tissue necrosis: induces release of marginated pool, bone marrow neutrophils (incl immature forms)
    • see a LEFT SHIFT: decreased Fc receptors (CD 16)
  • monocytosis: incr circulating monocytes due to chronic infl state and/or malignancy
  • eosinophillia: incr circulating eosinophils due to allergic rxn, parasitic infection, HL
  • basophilia: incr circulating basophils (classic: CML)
  • lymphocytic leukocytosis: incr circulating lymphocytes (viral infection, Bordetella pertussis inf)
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3
Q

infectious mononucleosis

[p54 - didn’t type it all out, this card is just here as placeholder for that section]

A
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4
Q

ACUTE LEUKEMIA

ALL

A

acute lymphoblastic leukemia

  • neoplastic accumulation of lymphoblasts (>20%) in bone marrow
    • positive nuclear staining for TdT (DNA polymerase), which is seen in lymphoblasts (but not myeloid blasts or mature lymphocytes)
  • most commonly in KIDS
    • assoc with Down syndrome over age 5
  • two subtypes:
    1. B-ALL (most common)
      • CD10, CD19, CD20
      • good response to response to chemo
      • t 12;21 more in kids, good prognosis
      • t 9;22 more in adults, poor prognosis
    2. T-ALL
      • CD2-CD8, but no CD10
      • teenagers presenting with thymic/mediastinal mass
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5
Q

ACUTE LEUKEMIA

AML

A

neoplastic accumulation of MYELOBLASTS

  • Neoplastic accumulation of immature myeloid cells (>20%) in the bone marrow
  • MPO+ (positive cytoplasmic staining for myeloperoxidase) → crystal aggregates visible as Auer rods
  • commonly in older adults
  • key subtypes:
    1. APL
    2. acute monocytic leukemia
    3. acute megakaryoplastic leukemia
  • may arise from pre-existing dysplasia, esp with prior exposure to alkylating agents or radiotx
    • Myelodysplastic syndromes present with cytopenias, hypercellular bone marrow, abnormal maturation of cells, incr blasts (<20%)
    • most pt die from infection or bleeding, some progress to acute leukemia
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6
Q

ACUTE LEUKEMIA

Acute Promyelocytic Leukemia: APL

A

acute promyelocytic leukemia

  • t (15;17) - translocation of RAR (chr17) to chr15
  • RAR disruption blocks maturation → promyelocytles accumulate
    • abnormal promyelocytes contain primary granules → incr risk for DIC
  • tx: ATRA (all trans retinoic acid) : binds altered receptor, causing blasts to mature and die
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7
Q

ACUTE LEUKEMIA

AML

acute monocytic leukemia

A
  • proliferation fo monoblasts (usually lack MPO)
  • characteristically infiltrate GUMS
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8
Q

ACUTE LEUKEMIA

AML

acute megataryoblastic leukemia

A
  • proliferation fo megakaryoblasts (lack MPO)
  • assoc with Downs syndrome before age 5
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9
Q

CHRONIC LEUKEMIA

basics

A
  • neoplastic prolif of MATURE circulating lymphocytes
  • characterized by high WBC count
  • insidious in onset
  • seen in older adults
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10
Q

chronic lymphocytic leukemia (CLL)

A

​CHRONIC LEUKEMIA

  • neoplastic prolif of naive B cells (coexpress CD5, CD20)
  • increased lymphocytes and smudge cells on blood smear
  • involvement of lymph nodes → generalized lymphadenopathy
    • aka “small lymphocytic leukemia”
  • complications:
    • hypogammaglobulinemia (bc neoplastic B cells dont produce Ig like normal B cells would)
    • autoimmune hemolytic anemia
    • transformation to diffuse large B cell lymphoma (Richter transformation)
      • marked by enlarging lymph node, spleen

most common leukemia overall

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11
Q

hairy cell leukemia

A

CHRONIC LEUKEMIA

  • neoplastic proliferation of mature B cells characterized by hairy cytoplasmic processes
  • cells positive for TRAP (tartrate resistant acid phosphatase)
  • clinical features:
    • splenomegaly (due to accum of hairy cells in red pulp)
    • “dry tap” on bone marrow aspiration (due to marrow fibrosis)
    • LYMPHADENOPATHY ABSENT!
  • excellent response to cladribine/2CDA (adenosine deaminase inhibitor) → causes adenosine to accumulate to toxic levels in neoplastic B cells
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12
Q

adult T cell leukemia

A

CHRONIC LEUKEMIA

  • neoplastic proliferation of mature CD4 T cells
  • associated with HTLV1
    • commonly seen in Japan, Caribbean
  • clinical features
    • rash (skin infiltration)
    • generalized lymphadenopathy with hepatosplenomegaly
    • lytic bone lesions with hypercalcemia
      • consider this as ddx to multiple myeloma, esp if see skin rashes too!
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13
Q

mycosis fungoides

A

CHRONIC LEUKEMIA

  • neoplastic proliferation of mature CD4 T cells
  • infiltrate skin, producing localized rash, plaques, nodules
    • aggregates of neoplastic cells in epidermis: Pautrier microabscesses
  • ​cells can spread to involve blood → Sezary syndrome
    • ​characteristic lymphocytes with cerebriform nuclei (Sezary cells) on smear
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14
Q

MYELOPROLIFERATIVE DISORDERS

basics

A
  • neoplastic proliferation of mature cells of myeloid lineage
  • avg age: 50-60
  • see high WBC count, hypercellular bone marrow
    • cells of all myeloid lineages increased
    • classify based on dominant myeloid cell produced
  • complications
    • incr risk for hperuricemia and gout (high turnover of cells/nuclei)
    • progression to marrow fibrosis or transformation to acute leukemia
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15
Q

chronic myeloid leukemia

A

MPD

  • neoplastic prolif of mature myeloid cells, esp GRANULOCYTES AND PRECURSORS (basophils characteristically increased
  • t (9;22) → Philadelphia chromosome = BCR-ABL fusion protein with increased tyr kinase activity
    • ​tx: imatinib (blocks BCR-ABL tyr kinase activity)
  • splenomegaly common
    • spleen enlarging? suggests progression to accelerated phase of disease, followed rapidly by transformation to acute leukemia
    • transformation to…
      • AML (2/3 cases)
      • ALL (1/3 cases)

how can you differentiate CML from a leukemoid rxn?

CML will show…

  • negative for LAP (leukocyte alkaline phosphatase, which will be present in granulocytes in a leukemoid rxn)
  • incr basophils
  • t(9;22)
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16
Q

polycythemia vera

A

MPD

  • neoplastic proliferation of mature myeloid cells, esp RBCS (granulocytes and platelets also incr)
  • associated with JAK2 kinase muatations
  • clinical symptoms (mostly due to hyperviscosity of blood)
    • blurry vision, headache
    • flushed face/congestion (plethora)
    • incr risk of venous thrombosis
    • itching/pruritis after bathing (bc of extra mast cells releasing histamine)
  • treatment:
    • 1st line: phlebotomy
    • 2nd line: hydroxyurea
    • w/out tx, death within a year

how do you distinguish PV from reactive polycythemia?

look at SaO2 and levels of epo

  • PV: low epo, normal SaO2
  • reactive polycythemia (high alt/lung disease): high epo, low SaO2
  • reactive polycythemia (ectopic epo production): high epo, normal SaO2
17
Q

essential thrombocythemia

A

MPD

  • neoplastic proliferation of mature myeloid cells, esp platelets (RBCs, granulocytes also incr)
  • assoc with JAK2 kinase mutation
  • symptoms realted to incr risk of bleeding and/or thrombosis
    • rarely progresses to marrow fibrosis or acute leukemia
    • no significant risk for hyperuricemia or gout
18
Q

myelofibrosis

A

MPD

  • neoplastic prolif of mature myeloid cells, esp megakaryocytes
  • assoc with JAK2 kinase mutation (50%)
  • megakaryocytes produce excess PDGF → causes marrow fibrosis
  • clinical features
    • splenomegaly due to extramedullary hematopoiesis
    • leukoerythroblastic smear (tear drop RBC, nucleated RBC, immature granulocytes)
    • incr risk of infection, thrombosis, bleeding
19
Q

LYMPHADENOPATHY

basics

A

enlarged lymph nodes

  • painful? lymph nodes draining region of acute infection
  • panless? chronic infl, metastatic carcinoma, lymphoma
    • inflammationL LAD due to hyperplasia of particular regions of lymph node

regions of lymph node:

  1. follicular hyperplasia (B cell region): ex. rheumatoid arthritis, early HIV
  2. paracortex hyperplasia (T cell region): ex. viral inf
  3. hyperplasia of sinus histiocytes: lymph nodes draining a tissue with cancer
20
Q

LYMPHOMA

basics

A
  • neoplastic proliferation of lymphoid cells that forms a mass
  • can arise in lymph node or extranodal tissue
  • two types
    • nonHodgkin lymphoma
      • classified based on B vs T, cell size, pattern of growth, expression of surface markers, cytogenetic translocations
      1. small B cells (follicular, mantle cell, marginal zone, small lymphocytic lymphoma)
      2. intermediate size B cells (Burkitt lymphoma)
      3. large B cells (diffuse large B cell lymphoma)
    • Hodgkin lymphoma
      • subtypes
      1. nodular sclerosis
      2. mixed cellularity
      3. lymphocyte rich
      4. lymphocyte depleted
21
Q

follicular lymphoma

A

NHL: small B cell (CD20)

  • neoplastic prolif of small B cells that form follicle like nodules
  • late adulthood w/ painless LAD
  • t(14;18) → BCL2 to IgH locus on chr14
    • overexpression of Bcl2 (anti-apoptotic)
    • ​​tx: low dose chemo or rituximab (only for symtomatic pts)
  • complicaation: progression to diffuse large B cell lymphoma: presents as enlarging lymph node

how do you tell the different between follicular lymphoma and reactive follicular hyperplasia?

follicular lymphoma shows…

  • disruption of lymph node architecture
  • lack of tingible body macrophages in germinal centers (which would be present in regular hyperplasia)
  • Bcl2 expression
  • monoclonality
22
Q

mantle cell lymphoma

A

NHL: small B cells (CD20)

  • neoplastic proliferation of small B cells expanding into mantle zone
  • late adulthood w/ painless LAD
  • t(11;14) → cyclin D1 to IgH locus on chr14
    • ​overexpression of cyclin D1 promotes G1/S transition → neoplastic prolif
23
Q

marginal zone lymphoma

A

NHL: small B cell (CD20): post-germinal center B cells

  • neoplastic proliferation of small B cells expanding into marginal zone
  • assoc with chronic infl states (Hashimoto thyroiditis, Sjogren syndrome, H pylori gastritis)
  • MALToma: marginal zone lymphoma formed in mucosal sites
    • miiiight regress with treatment of H pylori
24
Q

Burkitt lymphoma

A

NHL: intermediate B cells (CD20)

  • neoplastic proliferation of intermediate sized B cells
  • assoc with EBV
  • classic presentation: extranodal mass in child or young adult
    • African form: JAW
    • sporadic form: ABDOMEN
  • translocations of c-myc → overexpression of c-myc oncogene promotes cell growth
    • ​t(8;14) most common
  • ​high mitotic index, “starry sky” appearance
25
Q

diffuse large B cell lymphoma

A

NHL: large B cell (CD20)

  • neoplastic proliferation of large B cells growing diffusely in sheets
  • most common form of NHL
  • clinically aggressive (high grade)
  • arises sporadically or from transformation of a low grade lymphoma (ex. follicular lymphoma)
  • presents in late adulthood as enlarging lymph node or extranodal mass
26
Q

Hodgkin lymphoma

basics

4 subtypes

A
  • neoplastic proliferation of Reed Sternberg cells (large B with multilobed nuclei, prominent nucleoli, CD15, CD30)
    • secrete cytokines
      • sometimes see B symptoms (fever, chills, wt loss, night sweats)
      • attract reactive lymphocytes, plasma cells, macrophages, eosinophils
      • can lead to fibrosis
  • reactive inflammatory cells make up bulk of tumor - used as basis for classification
  1. nodular sclerosis
    • most common subtype (70%)
    • classic presentation: young adult female with enlarging cervical/mediastinal lymph node
    • lymph node divided by bands of sclerosis
    • RS cells present as lacunar cells
  2. mixed cellularity
    • assoc with abundant eosinophils (RS cells make IL5)
  3. lymphocyte rich
    • best prognosis
  4. lymphocyte depleted
    • most aggressive, usually in elderly and/or HIV+
27
Q

multiple myeloma

A

plasma cell disorder

  • malignant proliferation of plasma cells in bone marrow
    • most common primary malignancy of bone
    • high serum IL6 can be present → stimulates plasma cell growth and Ig production
  • have increased number of large plasma cells that are abnormal
    • plasma cells have nucleus on edge of cell
      • can be binucleated
  • clinical features
    • bone pain with hypercalcemia: lytic, punched out skeletal lesions, esp vertebrae/skull
      • ​neoplastic plasma cells activate RANK receptor on osteoclasts → bone destruction
    • elevated serum protein: M spike on SPEP
      • ​most common due to IgG, IgA
    • increased risk of infection
    • Rouleaux formation (incr serum protein → decr charge between RBCs)
    • primary AL amyloidosis: free light chains circulate in serum, deposit in tissues
    • proteinuria: free light chain excreted as Bence Jones protein → risk for renal failure (myeloma kidney)
28
Q

monoclonal gammopathy of undetermined significance (MGUS)

A
  • increased serum protein with M spike on SPEP but no other features of multiple myeloma
  • common in elderly
  • 1% of MGUS patients progress to multiple myeloma each year
29
Q

Waldenstrom macroglobulinemia

A
  • B cell lymphoma with monoclonal IgM production
  • clinical features
    • generalized LAD
    • NO LYTIC BONE LESIONS
    • incr serum protein with M spike (IgM)
    • visual, neuro deficits (ex. retinal hemorrhage/stroke) bc IgM causes hyperviscosity
    • bleeding: viscosity → defective platelet aggregation
  • complications treated with plasmapheresis to remove IgG from serum
30
Q

Langerhans cell histiocytosis

A

Langerhans cells: specialized dendritic cells found predominantly in skin

  • derived from bone marrow monocytes
  • present antigen to naive T cells

neoplastic proliferation of Langerhans cells

  • characteristic Birbeck granules (tennis racket)
  • cells CD1a+ and S100+ on immunohistochem
31
Q

Letterer-Siwe disease

A

Langerhans cell histiocytosis

  • malignant prolif of Langerhans cells
  • classic presentation: under 2 yrs
    • skin rash
    • cystic skeletal defects
  • multiple organs can be involved, rapidly fatal
32
Q

eosinophilic granuloma

A

Langerhans cells histiocytosis

  • benign proliferation of Langerhans cells in bone
  • classic presentation: pathologic fracture in adolescent
    • skin not involved
  • biopsy: Langerhans cells with mixed infl cells (numerous eosinophils)
33
Q

Hand-Schuller-Christian disease

A

Langerhans cells histiocytosis

  • malignant proliferation of Langerhans cells
  • classic presentation: child
    • scalp rash
    • lytic skull defects
    • diabetes insipidus
    • exophthalmos

Heme/Onc Pathoma (10 decks)

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