WBC disorders Flashcards

1
Q

Acute Leukemia

A
  • neoplastic proliferation of blasts (> 20% blasts in BM)
  • increased blasts “crowd out” normal hematopoiesis–> anemia, bleeding, infection
  • acute onset
  • blasts leak out into blood (large, immature cells with punched out nucleoli on blood smear)
  • divided into ALL(TdT+) and AML (MPO+)
  • high WBC
  • younger population
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2
Q

B-ALL

A
  • B cell Acute Lymphoblastic Leukemia
  • neoplastic proliferation of lymphoblasts
  • most common form of childhood leukemia
  • TdT (+)
  • CD10, 19,20
  • good prognosis for kids
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3
Q

T-ALL

A
  • T-cell Acute Lymphoblastic Leukemia/Lymphoma
  • TdT (+)
  • CD2-8
  • T for teenagers and thymic mass (mediastinal adenopathy)
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4
Q

chronic leukemia

A
  • neoplastic proliferation of mature lymphocytes
  • usually insidious onset; adults
  • CLL, Hairy cell leukemia, ATLL, mycosis fungoides
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5
Q

CLL

A
  • Chronic Lymphocytic Leukemia
  • lymphocytosis (hence the name! remember it’s a proliferation of mature B cells)
  • involvement of LNs = small lymphocytic lymphoma (SLL)
  • widespread lymphadenopathy + liver and spleen enlargement (unlike diffuse large B cell lymphoma)
  • neoplastic proliferation of Naive B cells – antibody production is all screwed up –> hypogammaglobulinemia and autoimmune hemolytic anemia
  • CD20 and CD5 (normally expressed on T cells)
  • older adults; most common neoplasm overall
  • smudge cells on blood smear
  • Richter transformation: transform to diffuse large B-cell lymphoma (enlarging LN or spleen)
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6
Q

Hairy Cell Leukemia

A
  • neoplastic proliferation of mature B cells characterized by hairy cytoplasmic processes
  • TRAP and TAP: TRAP stain, get trapped in spleen (no lymphadenopathy), “dry tap” (marrow fibrosis), trapped in hair
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7
Q

ATLL

A
  • Adult T Cell Leukemia
  • neoplasm of mature T cell
  • associated with HTLV-1 infection (Japan and Caribbean)
  • rash
  • hypercalcemia (lytic punched out bone lesions)
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8
Q

Mycoses Fungoides

A
  • neoplastic proliferatrion of mature CD4+ T cells that infiltrate skin –> skin rash, plaques and nodules
  • Sezary cells: lymphocytes with cerebriform nuclei

Sezary and Mycoses Fungoides are very fancy

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9
Q

myeloproliferative disorders

A
  • neoplastic proliferation of mature cells of myeloid lineage (the mature version of AML)
  • CML, PV, ET, myelofibrosis
  • late adulthood
  • high WBC with hypercellular BM (cells of all myeloid lineages are increased but disease defined by the predominant type)
  • increased risk for hyupericemia and gout (due to increased cell turnover)
  • progression to myelofibrosis (“burn out”) or AML (“blast out”)
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10
Q

CML

A
  • Chronic Myeloid Leukemia
  • neoplastic proliferation of myeloblasts (neutrophils, eosinophils and basophils)
  • Philly Chromo t(9;22) —BCR-ABL fusion protein (Philly CreaML cheese)
  • can transform to AML or ALL (“blast crisis”) since mutation is in pluripotent stem cell
  • low LAP as a result of low activity in mature granulocytes (vs. leukemoid rxn)
  • responds to imatinib (blocks tyr kinase actvity of fusion protein)
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11
Q

polycythemia vera

A
  • neoplastic proliferation of mature myeloid cells, esp RBCs
  • hematocrit > 60%
  • JAK2 kinase mutation
  • hyperviscous blood: blurry vision and HA, venous thrombosis (Budd-Chiari)
  • intense itching after hot shower
  • hyperuricemia and gout (due to rapid cell turnover)
  • distinguished from reactive polycythemia by low EPO and normal SaO2
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12
Q

AML

A
  • acute myeloid leukemia
  • myeloblasts characterized by MPO and Auer rods
  • subtypes include APL, AMoL, AMegL
  • may arise from pre-existing myeloproliferative disorders
  • risk factors: exposure to alkylating agents, chemo, radiation
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13
Q

APL

A
  • Acute Promyelocytic Leukemia/ M3 (subtype of AML)
  • MPO + Auer rods
  • t(15;17) —- translocation of RAR –> RAR disruption blocks maturation and promyelocytes/blasts accumulate
  • tx: all-trans RA (vit A)
  • DIC: high concentration of Auer rods released upon chemotx
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14
Q

ET

A
  • Essential Thrombocytopenia
  • neoplastic proliferation of mature myeloid cells, esp platelets
  • JAK2 mutation
  • increased risk of bleeding (dysfunctional platelets) and/or thrombosis (hyperactive platelets)
  • throbbing, burning pain in extremities caused by clotting of arterioles
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15
Q

Myelofibrosis

A
  • neoplastic proliferation of mature myeloid cells, esp megakaryocytes
  • megakaryocytes produce PDGF –> marrow fibrosis
  • splenomegaly due extramedually hematopoeisis
  • tear-drop RBCs (RBCs squeezed out of fibrotic marrow)
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16
Q

Follicular Lymphoma

A
  • neoplastic proliferation of small B cells (CD20+) that form follicle-like nodules
  • painless lymphadenopathy
  • t(14:18) bcl-2 activation
  • disruption of normal LN architecture with many follicles (as opposed to reactive follicular hyperplasia)
  • can progress to diffuse large B-cell lymphoma (enlarging LN)

Fourteen starts with F t(14;18)

17
Q

Mantle Lymphoma

A
  • neoplastic proliferation of small B cells (CD20+) that expands mantle zone (zone surrounding germinal center)
  • t (11;14) – cyclin D1 activation (“cycle the mantle”)

Mantlle = 11;14

18
Q

Burkitt Lymphoma

A
  • Neoplastic proliferation of B cells (CD20+)
  • young African children (jaw) and AIDS PTs associated with EBV
  • sporadic form in young adults (abdomen)
  • t (8;14) – c-myc activation
  • “starry sky” appearance (sheets of lymphos with interspersed macros)

B=8 t(8;14)

19
Q

diffuse large B-cell lymphoma

A
  • neoplastic proliferation of large B cells (CD20+)
  • localized disease – presents with enlarging LN or extranodal mass (Waldeyer ring)
  • arises sporadically or from transformation of follicular lymphoma
  • older adults
  • clinically aggressive (large cells are poorly differentiated)
20
Q

Hodgkin Lymphoma

A
  • neoplastic proliferation of Reed Sternberg cells (large B cells - CD15+)
  • RS cells secrete cytokines –> attract reactive cells (lymphocytes, plasma cells, macros) and “B” symptoms: fever, chills, weight loss, night sweats
  • nodular sclerosis is most common subtype (LN node divided by bands of sclerosis – creates nodules) –> enlarging cervical or mediastinal LN in young adult
  • lymphocyte rich type has best prognosis; lymphocyte depleted worst
  • mixed cellularity type characterized by eosinophils (RS cells secrete IL5)
  • 50% of cases associated with EBV
21
Q

multiple myeloma

A
  • malignant proliferation of plasma cells in BM
  • M spike: large amts of IgG or IgA
  • punched out lytic bone lesions (neoplastic cells activate osteoclasts)
  • increased risk of infection (monoclonal Ab lacks antigenic diversity)
  • primary amyloidosis (AL) – free light chain deposition and excretion in urine (Bence Jones proteinuria)
  • rouleaux formation of RBCs
22
Q

MGUS

A
  • Monoclonal Gammopathy of Undetermined Significance
  • plasma cell dyscrasia
  • isolated M spike w/o findings of MM
23
Q

Waldenstrom Macroglobulinemia

A
  • plasma cell dyscrasia
  • B-cell lymphoma with monoclonal IgM production (M spike due to IgM)
  • hyperviscosity symptoms

**IgM is very large – hence “macro” and viscosity

24
Q

Langerhans cell histiocytosis

A
  • proliferative disorder of dendritic cells in skin (present antigen to naive T cells)
  • child with lytic bone lesion and skin rash
  • Birbeck granules (“tennis rackets” on EM)