White Blood Cells Flashcards

Question

Adult T cell Leukemia/Lymphoma | Pathogenesis (2) Morphology (2) Presentation (5) Prognosis (2)

Answer 1

Adults with Human T cell Leukemia Retrovirus-1 (HTLV1) HTLV-1 encodes Tax protein that actives NFKB ``` Multilobated nuclei (cloverleaf/flower cells) CD4+ T cells ``` ``` Skin lesions Generalized lymphadenopathy Hepatosplenomegaly Peripheral blood lymphocytosis Hypercalcemia ``` If only skin, indolent If systemic, rapidly progressive, death within 1 year

Answer 2

CD4+ T cells CLA adhesion molecule CCR4 CCR10 Mycosis Fungoides: Phases: 1) Premycotic 2) Plaque 3) Tumor Neoplastic T cells in Epidermis and Upper Dermis Cerebriform appearnce to epidermis/dermis Late phase spread to lymph nodes and bone marrow Sezary Syndrome: Generalized exfoliative erythroderma Leukemia with Sezary cells showing cerebriform nuclei

Answer 3

STAT3 mutations Either T cells or NK cells Large lymphocytes with abundant blue cytoplasm Coarse azurophilic granules CD3 (T cells) CD56 (NK cells) Neutropenia Anemia Felty Syndrome: Rheumatoid arthritis, Splenomegaly, Neutropenia

Answer 4

Highly associated with EBV** Cell infiltrate surrounds small vessels, ischemic necrosis Large azurophilic granules Mostly NK cells, less T cells Destructive nasopharyngeal mass** Or mass in testes/skin Aggressive, responds well to radiation (no chemo)

Answer 5

Most common cancer of teenagers Usually presents in 30s Arises in Lymph Nodes Spreads to contiguous lymphoid tissues Spreads to Spleen, then Liver, then Bone marrow Activation of NFKB via: EBV (cells express LMP-1) IkB or A20 (TNFAIP3) mutations Good prognosis with radiation therapy

Answer 6

Reed Sternberg Cells: Multinucleated giant cells with inclusion-like nucleoli Variants: Mononuclear, Lacunar cells, Lymphohistiocytic PAX5 CD15 CD30 Painless Lymphadenopathy Fever, night sweats, weight loss (disseminated disease) Cutaneous immune anergy

Answer 7

Most common Not usually EBV associated Starts in Lower Cervical and Supraclavicular LN's Lacunar cells Few Reed Sternberg Cells Deposition of collagen that divides up lymph nodes

Answer 8

Older Males Plentiful Reed Sternberg cells Presents with Advanced tumor Very good prognosis

Answer 9

Reactive Lymphocytes Mononuclear cells Reed Sternberg cells Often EBV associated Good Prognosis

Answer 10

Least common Older adults HIV (+) people Reed Sternberg cells and their subtypes EBV associated 90% of time Least favorable prognosis

Answer 11

*Non-Classical HL Males under 35 ``` Lymphocytic and Histiocytic variant cells Multilobed nuclei (popcorn kernel cells) Expanded B cell follicles (nodular growth) ``` CD20 BCL6 Cerivcal and Axillary lymphadenopathy Can transform into Diffuse Large B cell lymphoma NOT EBV associated

Answer 12

AML with Genetic Aberrations: mostly good prognosis AML with MDS-like Features: poor prognosis Therapy-related AML: worst prognosis t(8;21) RUNX1/ETO fusion* inv(16) CBFB/MYH11 fusion* t(15;17) RARA/PML fusion (best prognosis) Karytotypic aberrations Mutations of transcription factors of myeloid differentiation Epigenetic alterations After alkylator, radiation or anti-Topoisomerase II therapy

Answer 13

Requires bone marrow biopsy showing at least 20% myeloid blasts Myeloblasts with peroxidase (+) azurophilic granules Auer rod granules Monoblasts that are esterase (+) CD33 and CD34 Anemia: fatigue Neutropenia: fever Thrombocytopenia: Bleeding, bruising, Petechiae Opportunistic infections Tissue Infiltration: Leukemia cutis (monocytic differentiation) Granulocytic sarcoma

Answer 14

Primary - idiopathic Secondary - genotoxic drugs or radiation Similar epigenetic factors to AML Mutations involving RNA splicing factors Transcription factor mutations TP53 mutations Myeloblasts < 20% Megaloblasts Ring sideroblasts Nuclear budding abnormalities: misshapen outlines Pseudo Pelger Huet cells (bilobed neutrophils) Pawn ball megakaryocytes Mean age 70 Pancytopenia Progression to AML

Answer 15

Mutated, constitutively activated Tyrosine Kinases Cause growth factor independence Increased bone marrow proliferation Increased extramedullary hematopoiesis Spent phase: marrow fibrosis, peripheral cytopenias Transformation to acute leukemia

Answer 16

Adult males Philadelphia Chromosome (9;22)(q34;q11) BCR-ABL fusion gene Constitutively activated ABL tyrosin kinase Originates from pluripotent HSC's Macrophages with wrinkled green-blue cytoplasm Hypercellular marrow Leukocytosis Decreased Leukocyte Alkaline Phosphatase (LAP) Insidious onset: mild anemia Splenomegaly: abdominal fullness or pain Hepatomegaly, lymphadenopathy Blast crisis during late accelerated phase

Answer 17

Activating JAK2 point mutation Causes upregulated RBC production Increased red cell, granulocyte and megakaryocyte precursors Spent phase: marrow fibrosis ``` Low EPO Elevated HCT Increased Red Cell Mass (hyperviscous blood) Leukocytosis Thrombocytosis ``` ``` Major bleeding episodes* Venous thrombosis* Hyperuricemia Spent stage May progress to AML ``` Treat with phlebotomy

Answer 18

Activating mutations in JAK2 Activating mutations in MPL Isolated elevated platelet count (thrombocytosis) Large platelets Elevated megakaryocyte count on bone marrow biopsy Erythromelalgia (burn/throb of hands/feet) Bleeding Thrombosis Gentle chemo to stop thrombopoiesis

Answer 19

Activating mutations in JAK2 Activating mutations in MPL Obliterative bone marrow fibrosis via non-neoplastic fibroblasts making collagen Caused by PDGF and TGF-Beta from megakaryocytes Normochomic normocytic Anemia Prominent splenomegaly Tear drop shaped RBC's (dacrocytes)* Leukoerythroblastosis* Poor prognosis, hard to treat Death via infections, bleeding abnormalities

Answer 20

BRAF point mutation Val-Glu ``` Birbeck granules (pentalaminar tubules) Abundant cytoplasm, nuclei with linear grooves ``` CCR6 CCR7

Answer 21

``` Seborrheic eruption cutaneous lesions Chronic ottitis media Mastoiditis Hepatosplenomegaly Lymphadenopathy Lung lesions Bone lesions ``` Rapidly fatal if untreated (50% survive if treated)

Answer 22

Langerhans cells mixed with eosinophils, lymphocytes, plasma cells, and neutrophils Arises in medullary cavity of calvarium, ribs, femur Unifocal painful bone lesions Indolent, may resolve spontaneously or with excision Multifocal erosive painful bone lesions Presents in young children Hand-Schuller-Christian Triad: Diabetes Insipidus, Exopthalmus, Calvarium defects

Answer 23

Chronic smoking | BRAF mutations