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****MS 2 Unit 2 Pathology > White Blood Cell Disorders > Flashcards

White Blood Cell Disorders Flashcards

1
Q

Hematopoiesis Overview

A
  • stepwise maturation of CD34+ hematopoietic stem cells

* cells mature and are released from marrow to blood

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2
Q

Leukopenia–> classifications and what causes them

A
  • <b>Neutropenia</b> due to drug toxicity (damages stem cells), severe infection (neutrophils migrate to tissues instead of staying in circulation); treatment: GM-CSF or G-CSF
  • <b>Lymphopenia</b>: due to immunodeficiency, high cortisol (apoptosis of lymphocytes), autoimmune destruction, radiation
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3
Q

Leukocytosis

A

<b>Neutrophilia</b> due to bacterial infection/tissue necrosis (release of marginated pool & bone marrow neutrophils including immature forms with decreased Fc receptors), high cortisol (impairs adhesion also causing release of marginated pool)

<b>Monocytosis</b> due to chronic inflammatory states and malignancy

<b>Eosinophilia</b> due to allergic reactions, parasitic infections and Hodgkins (via increased IL5); increased eosinophil chemotactic factor

<b>Basophilia</b> due to CML

<b>Lymphocytic leukocytosis</b> due to viral infections (hyperplasia of T cells) and Bordetella pertussis infection (bacteria produce a promoting factor which blocks lymphocutes from leaving blood to enter lymph node)

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4
Q

Infectious Mononucleosis (IM)–> cause, clinical features, mechanism, screening, complications

A
  • causes: EBV (CD8+) or CMV
  • <b>EBV infects oropharynx, liver (causes hepatitis with hepatomegaly and elevated liver enzymes) and B-cells</b>
  • CD8 T-cell response–> hyperplasia in paracortex & PALS–> lymphadenopathy & splenomegaly respectively
  • <b>monospot test</b> detects IgM Ab that cross-react with heterophile Ab turning (+) 1 wk post infection ((-) could mean CMV is the cause); <b>serologic testing for EBV viral capsid antigen</b>
  • Complications: splenic rupture (avoid contact sports), rash, risk of B-cell lymphoma
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5
Q

Acute Leukemia Overview–> causes and types

A
  • neoplastic proliferation of blasts (large, immature, punched out nucleoli, little cytoplasm) <b>>20%</b>
  • crowds out normal hematopoiesis–> causes <b>anemia, thrombocytopenia or neutropenia</b>

<b>Acute Leukemia = ALL & AML</b>

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6
Q

Acute Lymphoblastic Leukemia (ALL)–> causes, types

A
  • accumulation of lymphoblasts (immature) that <b>stain (+) for TdT</b>
  • common in Down Syndrome patients >5yo

<b>B-ALL</b>: CD10, CD19, CD20; prophylaxis to scrotum and CSF; t(12;21) in children & t(9;22) in adults

<b>T-ALL</b>: CD2-CD8 (NO CD10); presents as mediastinal (thymic) mass called acute lymphoblastic lymphoma

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7
Q

Acute Myeloid Leukemia (AML)–> causes, types

A
  • accumulation of immature myeloid cells >20% in marrow that <b>stain (+) for MPO</b>
  • could arise from pre-exisiting dysplasia (myelodysplastic syndomes) from exposure to alkylating agents or radiotherapy

<b>Acute Promyelocytic Leukmia (APL)</b>: t(15;17) translocation of <b>retinoic acid receptor (RAR)</b> on X17 to X15; RAR disruption blocks promyelocyte maturation; increased risk for DIC due to primary granules of abnormal promyelocytes; treatment: ATRA

<b>Acute Monocytic Leukemia</b>: monoblast proliferation infiltrating gums; lacks MPO

<b>Acute Megakaryoblastic Leukemia</b>: megakaryoblastic proliferation associated with Down Syndrome

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8
Q

What do myelodysplatic syndromes usually present with?

A

-cytopenias, hypercellular bone marrow, abnormal maturation of cells, increased blasts (

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9
Q

Chronic Leukemia Overview

A
  • neoplastic proliferation of <b>mature</b> circulating lymphocytes (high WBC count)
  • slow, asymptomatic, in adults
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10
Q

Chronic Lymphocytic Leukemia (CLL)–> causes, complications

A
  • neoplastic proliferation of <b>naive</b> B-cells that co-express CD5 & CD20
  • increased <b>lymphocytes and smudge cells</b>
  • generalized lymphadenopathy

-<b>complications: hypogammaglobulinemia (risk of infections), autoimmune hemolytic anemia, Richter transformation (to diffuse large B-cell lymphoma)</b>

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11
Q

Hairy Cell Leukemia–> cause, features, treatment

A
  • neoplastic proliferation of <b>mature</b> B-cells characterized by hairy cytoplasmic processes
  • <b>(+) for TRAP</b>
  • splenomegaly (accumulations in red pulp–> do not go to nodes), “Dry tap” on marrow aspiration due to fibrosis
  • responds to <b>2-CDA (adenosine deaminase inhibitor)</b> since adenosine accumulates to toxic levels</b>
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12
Q

Adult T-Cell Leukemia/Lymphoma (ATLL)–> causes and features

A

-neoplastic proliferation of <b>mature CD4+ T-cells</b> associated with HTLV-1 (common in Japan & Caribbean)

<b>-rash, generalized lymphadeopathy with hepatosplenomegaly & lytic bone lesions with hypercalcemia</b>

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13
Q

Mycosis Fungoides

A
  • neoplastic proliferation of <b>mature CD4+ T-cells</b> that infiltrate the skin causing rash, plaques and nodules
  • <b>Pautrier microabscesses: aggregates of neoplastic cells in the epidermis</b>

<b>Sezary Syndrome</b>: when cells spread to involve the blood (Sezary cells= lymphocytes with cerebriform nuclei)

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14
Q

Myeloproliferative Disorders (MPD) Overview

A
  • neoplastic proliferation of mature cells of the myeloid lineage causing high WBC count
  • usually in older adults
  • <b>complications</b>: high turnover of cells increases risk of hyperuricemia/gout; progression to marrow fibrosis or transformation to acute leukemia
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15
Q

Chronic Myeloid Leukemia (CML)–> cause, what it leads to

A
  • neoplastic proliferation of mature myeloid cells (<b>especially granylocytes like basophils</b>)
  • t(9;22)–>Philadelphia–>bcl-abl fusion
  • <b>splenomegaly suggests accelerated phase</b>
  • 2/3 transform to AML, 1/3 to ALL</b>
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16
Q

CML vs. Leukemoid reaction (infection)

A

CML:
(-) LAP
-increased basophils
-t(9;22)

Leukemoid Reaction
(+) LAP
-no increase of basophils
-absent t(9;22)

17
Q

Polycythemia Vera (PV)

A
  • neoplastic proliferation of mature myeloid cell (<b>especially RBCs</b>); granulocytes and platelets are also increased
  • <b>JAK2 kinase mutation</b>
  • symptoms due to hyperviscosity of blood (blurry vision, increased risk of venous thrombosis, flushing, itching)

-treatment: phlebotomy, hydroxyurea

18
Q

Polycythemia Vera vs. Reactive Polycythemia

A

<b>PV</b>

  • decreased EPO
  • SaO2= normal

<b>RP (high alt/lung disease)</b>

  • increased EPO
  • SaO2= low

<b>RP (ectopic EPO production from renal cell carcinoma)</b>

  • increased EPO
  • SaO2= normal
19
Q

Essential Thrombocytopenia (ET)

A
  • neoplastic proliferation of mature myeloid cells (<b>especially platelets</b>); RBCs and granulocytes are also increased
  • <b>JAK2 kinase mutation</b>
  • megakaryocytes–> excess PDGF–> marrow fibrosis

-splenomegaly (extramedullary hematopoiesis), leukoerythroblastic smear, increased risk of infection, thrombosis and bleeding (marrow can’t properly produce cells and spleen can’t compensate)

20
Q

Lymphadenopathy (LAD) Overview

A

-enlarged lymph nodes

  • <b>painless</b>: nodes that are draining acute infection
  • <b>painful</b>: chronic inflammation, metastatic carcinoma or lymphoma
  • in inflammation, node enlargement is due to hyperplasia of particular regions<b>
    1. Follicular (rheumatoid arthritis, HIV)
    2. Paracortex (viral infections)
    3. Sinus Histiocytes (tissue with cancer- has become reactive)</b>
21
Q

Lymphoma Overview

A
  • neoplastic proliferation of lymphoid cells forming mass
  • non-Hodgkins & Hodgkins

-non-Hodgkins–> small B-cells (follicular, mantle cell, marginal zone, small lymphocytic), intermediate-sized B-cells (Burkitt lymphoma), large B-cells (B-cell lymphoma)

22
Q

Hodgkins vs. non-Hodgkins

A

<b>Hodgkins</b>

  • 40%
  • Reed-Sternberg cells
  • mass= reactive cells (inflammatory cells & fibrosis(
  • painless, B-symptoms
  • young adults
  • spread- contiguous; rarely extranodal
  • no leukemic phase

<b>non-Hodgkins</b>

  • 60%
  • Lymphoid cells
  • mass= lymphoid cells
  • painless
  • late adulthood
  • spread- diffuse, extranodal
  • leukemic phase
23
Q

Mantle Cell Lymphoma

A
  • neoplastic proliferation of <b>small B-cells</b> (CD20+) that expands the mantle zone
  • painless lymphadenopathy
  • <b>t(11;14)–> cyclin D1 on X11 translocates to Ig heavy chain locus on X14 causing its overexpression promoting G1/S</b>
24
Q

Marginal Zone Lymphoma

A
  • -neoplastic proliferation of <b>small B-cells</b> (CD20+) that expands the marginal zone (which is formed by post-germinal center B-cells)
  • associated with chronic inflammatory states (Hashimoto thyroiditis, Sjorden syndrome, H. pylori gastritis

-MALToma is in mucosal sites

25
Q

Follicular Lymphoma

A
  • neoplastic proliferation of <b>small B-cells</b> (CD20+) that form follicle-like nodules
  • late adulthood with painless lymphadenopathy
  • <b>t(14;18)</b>= BCL2 on X18 translocates to Ig heavy chain locus on X14–> BCL2 overexpression inhibiting apoptosis
26
Q

Follicular Lymphoma vs. Reactive Follicular Hyperplasia

A

<b>Follicular Lymphoma</b>

  • disruption of normal lymph node architecture
  • lack of tingible body macrophages in germinal centers
  • Bcl2 expression
  • monoclonality

<b>Reactive Follicular Hyperplasia</b>

  • no disruption of normal lymph node architecture
  • presence of tingible body macrophages in germinal centers
  • no Bcl2 expression
  • polyclonality
27
Q

Burkitt Lymphoma

A
  • neoplastic proliferation of <b>intermediate-sized B-cells</b> (CD20+)
  • associated with EBV
  • extranodal mass (African- jaw, Sporatic- abdomen)
  • <b>translocations of myc- t(8;14)</b>

<i>high mitotic index and “starry-sky” appearance on microscopy</i>

28
Q

Diffuse Large B-Cell Lymphoma

A
  • neoplastic proliferation of <b>large B-cells (CD20+)</b> growing in sheets
  • aggressive
  • <b>arises sporatically or from transformation of a low-grade lymphoma</b>
  • presents in late adulthood as an enlarging lymph node or extranodal mass
29
Q

Hodgkin Lymphoma (HL)

A
  • proliferation of Reed-Sternberg cells (large B cells with multilobed nuclei and prominent nucleoli (+) for CD15 & CD30)
  • secretion of cytokines causing B-symptoms, attraction of reactive cells and possible fibrosis

<b>Subtypes:</b>

  1. Nodular Sclerosis: most common, enlarging cervical/mediastinal lymph node with bands of sclerosis in young female
  2. Lymphocyte-Rich: best prognosis
  3. Mixed Cellularity: lots of eosinophils (RS produce IL5)
  4. Lymphocyte-Depleted: most aggressive in elderly & HIV positive patients</b>
30
Q

Name the 4 plasma disorders (Dyscrasias)

A
  1. Multiple Myeloma
  2. Monoclonal Gammopathy of Undetermined Significance (MGUS)
  3. Waldenstrom Macroglobulinemia
31
Q

Multiple Myeloma–> cause, clinical features

A
  • malignant proliferation of <b>plasma cells</b> in the marrow that is the most common primary malignancy of bone
  • <b>high IL6 stimulating plasma cells and Ig</b>
  • Clinical features:
  • <b>bone pain</b> (with hypercalcemia since plasma cells activate RANK receptor causing lytic lesions)
  • <b>elevated serum protein</b> (plasma cells produce Ig; M spike due to IgG or IgA)
  • <b>increased risk of infection</b> (monoclonal Ab lacks antigenic diversity)
  • <b>Rouleaux formation</b> (increased serum protein decreases charge between RBCs)
  • <b>primary AL amyloidosis</b> (increased free light chains circulate and deposit in tissues)
  • <b>proteinuria</b> (free light chain excreted as Bence Jones protein depositing in tubules- possible renal failure)
32
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS)

A

-increased serum protein with M spike
<b>-other features of Multiple Myeloma are absent </b>
-common in elderly
-1% will develop multiple myeloma

33
Q

Waldenstrom Macroglobulinemia–> cause, clinical features, treatment

A

-B-cell lymphoma with monoclonal IgM production

  • Clinical features:
  • generalized lymphadenopathy (no lytic bone lesions)
  • increased serum protein with M spike (IgM)
  • visual/neurologic deficits (IgM causes hyperviscosity)
  • bleeding (defective platelet aggregation)

*treatment: plasmapheresis (removed IgM)

34
Q

Langerhans Cells & Langerhans Cell Histocytosis Overview

A
  • Langerhans cells: specialized dendritic cells in the skin that present antigen to naive T cells (derived from bone marrow monocytes)
  • Langerhans Cell Histocytosis: neoplastic proliferation of Langerhans cells characterized by <b>Birbeck (tennis racket) granules; CD1+a and S100+</b>
35
Q

Letterer-Siwe Disease

A
  • malignant proliferation of <b>Langerhans Cells</b>

- skin rash, cystic skeletal defects in infant (

36
Q

Eosinophilic Granuloma

A
  • benign proliferation of <b>Langerhans Cells</b> in bone
  • in adolescents, presents as fracture (skin not involved)
  • <b>biopsy: Langerhans cells with mixed inflammatory cells with many eosinophils</b>
37
Q

Hand-Schuller-Christian Disease

A
  • malignant proliferation of <b>Langerhans cells</b>

- scalp rash, lytic skull defects, diabetes insipidus, exophthalmos in child (>3yo)

****MS 2 Unit 2 Pathology (4 decks)

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