LECOM WBC Path
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| Tennis rackets, Seborrheic eruptions, otitis media and mastoiditis | Langerhans cell histiocytosis
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| IgM Spike, hepatosplenmegaly and lacking hypercalcemia | lymphoplasmacytic lymphoma
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| Leukocytosis, immature myeloid cells in blood, increased leukocyte ALK PHOS | leukemoid reaction
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| Leukocytosis, immature myeloid cells in blood, low leukocyte ALK PHOS | CML
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| t(8;14), illeocecal mass, med. Leuks w/ coarse chromatin, high mitotic, starry sky | sporadic Burkitts
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| no HSM; Node/extranodal/tonsils large lymphoid cells large nuclei, CD 19, 20, 10 and BCL6 | DLCBL
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| High RBC(marked), platelets, & WBC; low erythropoietin | Polycythemia Vera
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| Polycythemia Vera “burns out” to | myelofibrosis
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| Polycythemia Ver ‘blast out” to | AML
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| Ringed sideroblasts, mafaloblasts, abnormal megakaryocytes, myeloblasts in marrow | myelodysplasia
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| Myelodysplasia with 5q deletion is caused by | post-therapy
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| High platelets &WBC, no marrow fibrosis, no polycythemia;large platelets | Essential Thrombocytosis
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| Virus associated w/ endemic Burkits lymphoma | EBV
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| Mass lesion in stomach, thyroid, or salivary, CD19/20+, CD3-, better after treatment | MALToma
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| <2yo, skin&pulmonary lesions, osteolytic lesion;Birbek granules | Letterer-Siwe Disease (Langerhans)
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| Typical cytoplasmic PAS + finding in AML | Auer Rod
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| anemia, thrombo/neutrocytopenia, marrow: delicate chromatin, nucleoli, az.granules | AML
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| Adult, cutaneous rash/lesions, numb, LA&HSM, anemic, lymphocytosis “clover leaf” cells | Adult TCL
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| Adult T-Cell Lymphoma/ Leukemia associated virus / countries | HTLV-1, Japan, W. Africa, Caribean
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| T or B Cell: CD1 | T-Cell
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| T or B Cell: CD3 | T-Cell
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| T or B Cell: CD4 | T-Cell
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| T or B Cell: CD5 | T-Cell
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| T or B Cell: CD8 | T-Cell
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| T or B Cell: CD10 | B-Cell
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| T or B Cell: CD19 | B-Cell
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| T or B Cell: CD20 | B-Cell
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| T or B Cell: CD21 | B-Cell
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| T or B Cell: CD23 | B-Cell
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| T or B Cell: CD79a | B-Cell
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| Lymphoid neoplasms mostly B or T cell? | B cell, 85-90%
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| BALL or TALL more common? | BALL 85%
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| Most common cancer of children? | ALL
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| ALL more common in what race? | hispanics
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| B-ALL age peak | 3yo
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| T-ALL age peak | “adolescence”
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| Mass present in 50-70% of T-ALLs | mediastinal thymic mass
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| ALL vs AML. ALL: ____chromatin, ______cytoplasm lacking ______ | difuse ; smaller amount; granules
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| ALL stains vs AML: myeloperoxidase ____ and cytoplasm PAS____ | myeloperoxidase negative; PAS +
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| Positive stain in 95% of both B-and T- ALL | TdT
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| TdT + and CD19+, CD10+ most likely Dx | B-ALL
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| Poor prognosis for ALL. Age? Genetics? | Under 2yo; peripheral blast >100k; t(9;22) Philadelphia chrm
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| Favorable prognosis for ALL. Age? Genetics? | 2-10yo; low WBC count; hyperploidy; trisomy 4,7,10; t(12;21)
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| Hyperdiploidy only seen in which? T-ALL or B-ALL? | B-ALL
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| GOF NOTCH1 mutation in T-ALL or B-ALL? | T-ALL
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| Philidelphia Chromosome # and fusion? | t(9;22); activated BRC-ABL gene
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| Most Common Cause of agranulocytosis | drug toxicity.
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| Common clinical manifestation of agranulocytosis | ulcerating, necrotizing lesions of the
oropharyngeal mucosa
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| agranulocytosis predisposes to these deep fungal infections | Candida and Aprergillus
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| Below ___/mm3 neutrophils, serious Infx are likely | 500
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| This IL stimulates mainly eosinophils | IL-5
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| This GF induces neutrophilia | G-CSF
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| In sepsis or sever inflammatory reactions, leukocytosis is usually accompanied by these morphologic changes in neutrophils | toxic granulations, Dohle Bodies, and cytoplasmic vacuoles
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| Sky blue cytoplasmic puddles of dilated ER | Dohle bodies
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| Coarse, markedly darker abnormal azurophilic granules | toxic granules
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| Causes of neutrophilia | Acute bacterial Infx, usually pyogenic; MI or burns
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| Causes of eosinophilia | allergic disorders; parasite Infx; drug reactions; collagen vascular disorders
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| Causes of basophilia | rare; CML
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| Causes of monocytosis | chronic INFx; rickettsiosis, malaria; SLE; IBD
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| Causes of lymphosytosis | Viral Infx; pertussis; w/ monocytosis in chronic immunological stimulation (Tb)
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| t(9,22) is associated with what disease(s)? | ALL, AML, CML
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| what inherited genetic factors predispose to WBC neoplasias? | Bloom syndrome, Fanconi anemia, ataxia telangiectasia = Acute leukemias
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| Inherited genetic condition predisposing to childhood leukemias | +21, NF-1
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| Which viruses predispose to WBC neoplasias? | HTLV-1, EBV, KSHV
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| Virus associated with adult T cell leukemia/lymphoma | HTLV-1
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| Virus associated with Burkitt lymphoma, Hodgkin lymphoma, other B cell lymphomas in setting of T-cell immunodeficiency and NK-cell lymphomas | EBV
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| Virus associated with Kaposi sarcoma | KSHV
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| what environmental agent is associated with gastric B-cell lymphomas? | H. pylori infection
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| chronic immune stimulation in what disease is associated with intestinal T-cell lymphomas? | gluten-sensitive enteropathy
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| which plasma cell neoplasm often presents as pain d/t pathologic fractures? | multiple myeloma
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| which WBC neoplasms cause symptoms through the secretion of circulating factors? | plasma cell tumors (secretion of antibodies or fragments)
Hodgkin lymphoma (fever d/t release of inflammatory cytokines)
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| MCC of cancer in children? | ALL
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| which subset of ALL presents as childhood leukemia? | B-ALL
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| which lymphoid neoplasm presents in adolescent males as thymic lymphomas (mediastinal mass)? | T-ALL
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| lymphoblasts express CD19 and karyotype shows hyperdiploidy and t(12,21)? | B ALL
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| lymphoblasts often express CD1a and gain-of-fxn mutations in NOTCH1? | T ALL
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| which WBC neoplasm causes compression of large vessels and airways in the mediastinum? | T-ALL
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| which drug is used in t(9,22)+ ALLs and inhibits BCR-ABL kinase? | Imatinib (Gleevec)
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| ALL responsiveness to aggressive chemo (including CNS prophylaxis)? | CR in 90% of children, 2/3 cured
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| MCC of leukemia of adults in Western world? | CLL
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| difference between CLL and SLL? | degree of peripheral blood lymphocytosis
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| WBC neoplasm in which abnml cells gather in LNs in proliferation centers; PBS contains smudge cells? | CLL/SLL
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| Neoplastic cells are positive for CD19, CD20, CD23, and CD5; deletions in 13q, 11q, 17p, trisomy 12q? | CLL/SLL
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| poor prognosis in CLL/SLL is associated with what progression of disease? | prolymphocytic transformation (worsening cytopenias, inc splenomegaly, inc # prolymphocytes)
Richter syndrome - transformation to diffuse large B-cell lymphoma (rapidly enlarging mass in LN or spleen)
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| tumor that arises from germinal center B cells and is strongly assoc'd w/ translocations involving BCL2 |
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| t(14,18)]? | Follicular lymphoma
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| LN findings in follicular lymphoma? | centrocytes - small, cleaved cells
centroblasts - larger cells w/ open chromatin, several nucleoli, modest amts of cytoplasm
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| what are the results of t(14,18) mutation? | BCL2 and Ig heavy chain loci switch, BCL2 is overexpressed, promotes survival of follicular lymphoma cells by blocking bax channel
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| MCC of NHL in United States? | follicular lymphoma
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| response of follicular lymphomas to chemo/rad? | survival is not improved by aggressive therapy
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| mature B-cell tumors that express CD19, CD20 and sometimes translocation of BCL6; typically present at extranodal sites (GI, skin, bone, brain); aggressive, rapidly proliferating; may respond to therapy? | Diffuse large B-cell lymphoma
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| neoplasm with medium sized lymphocytes, "starry-sky" appearance, c-MYC t(8,14)? | Burkitt lymphoma
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| B-cell neoplasm assoc'd with EBV, presents in maxilla or mandible, involves kidneys, ovaries, adrenal glands? | African (endemic) Burkitt lymphoma
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| B-cell neoplasm with high incidence in AIDS, presents as mass involving ileocecum or peritoneum? | American (sporadic) Burkitt lymphoma
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| neoplasm of older adults presenting with hypercalcemia, bone pain, pathologic fxrs; inc gamma proteins, Bence-Jones proteins, and "punched-out" lesions of skull? | Multiple myeloma (plasma cell myeloma)
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| what causes lytic bone lesions in multiple myeloma? | inc osteoclast activating factor (OAF, IL-6)
MIP1a induces expression of RANKL which activates osteoclasts
Modulators of Wnt pathway inhibit osteoblasts
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| Disorder in which protein casts in DCT and collecting ducts are surrounded by multinucleated giant cells? | myeloma kidney
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| MCC of death in multiple myeloma? | infection (inc susceptibility d/t dec production of serum Ig's)
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| most common plasma cell dyscrasia? | monoclonal gammopathy of uncertain significance (MGUS)
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| MCC of elevated serum M protein level (< 3gm/dL) in asymptomatic older patient? | MGUS
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| B-cell neoplasm that infiltrates many organs, has M spike (IgM), but no hypercalcemia, and no lytic bone lesions present? | Lymphoplasmacytic lymphoma
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| what are Russell bodies and Dutcher bodies? | Russell bodies - cytoplasmic immunoglobulin
Dutcher bodies - intranuclear immunoglobulin
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| characteristic of lymphoplasmacytic lymphoma | hyperviscosity syndrome
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| hyperviscosity syndrome causes what complications? | vascular dilations and retinal hemorrhages --> visual problems
neuro sxs (HA, confusion)
abnml globulins cause bleeding and cryoglobulinemia (precipitate at low temp and cause Raynaud phenomenon)
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| lymphoid neoplasm of older males consisting of small, cleaved lymphocytes, but no proliferation centers, no large centroblasts; t(11,14), high levels of cyclin D, CD5+, CD23-? | Mantle cell lymphoma
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| B-cell tumor that commonly occurs at sites of chronic immune or inflammatory reactions? | marginal zone lymphomas (MALTomas)
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| Disease of middle-aged white males, may cause "dry tap" BM aspirate, presents with red beefy splenomegaly d/t infiltration of red pulp; stains positive for tartrate-resistant acid phosphatase (TRAP) | Hairy cell leukemia
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| neoplasm of CD4+ T-cells in adults infected with HTLV-1, esp. in Japan, W Africa, Caribbean; presents w/ skin lesions, hypercalcemia, enlarged LN, liver, and spleen; cloverleaf nuclei? | Adult T-cell Leukemia/Lymphoma (ATLL)
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| tumor of CD4+ T-cells that causes generalized pruritic erythematous rash (w/o hypercalcemia); abnml cells have cerebriform nuclei? | Mycosis fungoides/Sezary syndrome
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| difference btwn MF and Sezary syndrome? | skin lesions of sezary syndrome rarely become tumors
sezary cells (cerebriform nuclei) are found in peripheral blood in sezary syndrome, only found in epidermis/dermis in MF
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| rare neoplasm of CD8 T-cells, lymphocytes have abundant cytoplasm with coarse azurophilic granules? | Large granular lymphocytic leukemia
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| neoplasm presenting as descructive midline mass involving nasopharynx, skin, or testes; highly associated with EBV? | extranodal NK/T-cell lymphoma
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| how is HD different from NHL? | - presents with fever
- spread is to contiguous (adjacent) nodal groups
- characterization based on inflammatory response instead of malignant cell
- no leukemic state
- extranodal spread uncommon
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| neoplastic cell of this disease has "owl eye" bilobed nucleus w/ central nucleoli surrounded by clear space | Hodgkin lymphoma (Reed-Sternberg cells)
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| HL variant that has L&H "popcorn" cells and are negative for CD15 and CD30, but positive for B-cell markers CD20 and BCL6? lymphocyte predominance type |
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| most common type of HL; has lacunar variant Reed-Sternberg cells that are positive for PAX5, CD15 and CD30; nuclear deposition of collagen in bands? | Nodular sclerosis type
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| type of HL with infiltrate of T-cells, eosinophils, plasma cells, benign macrophages with Reed-Sternberg cells; RS cells often infected with EBV? | Mixed-cellularity type
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| type of HL with few lymphocytes, many RS cells? | lymphocyte depleted type
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| stereotyped progression of spread of HL? | nodal dz --> splenic dz --> hepatic dz --> marrow and other tissues
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| clinical presentations of HL? | usually painless lymphadenopathy
disseminated dz, mixed-cellularity or LD types may have B-cell symptoms: waxing/waning fever, wt loss, night sweats
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| population at risk for AML? | adults
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| dx of AML is based on what? | >= 20% myeloid blasts in bone marrow
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| many myeloblasts on PBS, auer rods, peroxidase+ azurophilic cytoplasmic granules - Dx? | AML
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| auer rods are especially pronounced in what neoplasia (specific translocation)? | t(15,17) - acute promyelocytic leukemia
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| treatment of M3 subtype AML? | all-trans retinoic acid (ATRA) - induces differentiation of myeloblasts
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| difference between acute leukemias and chronic leukemias? | mostly blasts (acute), more mature cells (chronic)
children or elderly (acute), midlife (chronic)
short and drastic (acute), longer, less devastating (chronic)
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| karyotypic abnormalities associated with AML? | de novo: t(8;21), inv(16), t(15;17)
following MDS or exposure to DNA-damaging agents: del or monosomies of 5 & 7
after tx w/ topoisomerase II inhibitors: MLL chromosome (11q23)
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| disorder associated with t(8;14)? | burkitt lymphoma (c-myc activation)
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| disorder associated with t(14;18)? | follicular lymphomas (bcl-2 activation)
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| disorder associated with t(15;17)? | M3 type AML
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| disorder associated with t(11;22)? | Ewing's sarcoma
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| disorder associated with t(11;14)? | Mantle cell lymphoma
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| most common presentation of AML? | sxs related to anemia, neutropenia, TCpenia (fatigue, fever, spontaneous mucosal and cutaneous bleeding)
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| what factors contribute to bleeding diathesis in AML? | thrombocytopenia
procoagulants and fibrinolytic factors released by leukemic cells (esp in t
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| 15;17]) |
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| which types of AML are high-risk? | those that follow MDS or genotoxic therapy or that occur in elderly
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| primary and t-MDS are associated with what karyotypic abnormalities? | monosomy 5 and 7;
del 5q, 7q, 20q; trisomy 8
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| most common finding in MDS? | dysplastic differentiation affecting all three myeloid lineages (erythroid, granulocytic and megakaryocytic)
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| morphological changes seen in MDS? | erythroid: ringed sideroblasts, megaloblastoid maturation, nuclear budding abnormalities
granulocytic; neutrophils with dec or abnml/toxic granules, pseudo-Pelger-Huet cells
Megakaryocytic: pawn ball meg.cytes
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| what are pseudo-Pelger-Huet cells? | neutrophils with only two lobes (seen in MDS)
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| primary MDS affects what age group? | > 60 y
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| what are the chronic myeloproliferative disorders? | - CML
- polycythemia vera
- essential thrombocytosis
- myelofibrosis w/ myleoid metaplasia
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| what features are common to all MPDs? | - neoplastic stem cells home in to secondary hematopoietic organs, esp spleen, leading to splenomegaly
- terminal spent phase: marrow fibrosis and peripheral cytopenias
- progression to acute leukemia
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| what is the biomechanical change that occurs in CML? | BCR gene (22) fuses with ABL gene (9) and synthesizes a constitutively active tyr-kinase, stimulating proliferation and survival of marrow progenitors
does not interfere with differentiation --> increase in mature cells in blood, esp granulocytes and plt
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| what lab finding helps differentiate between CML and other leukemoid reactions? | absence of leukocyte alk phos in CML
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| cell counts in CML - RBCs, WBCs, Plts? | dec RBCs
inc WBCs
inc Plts
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| pharm tx of CML? | Imatinib (anti-bcr-abl antibody)
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| disease characterized by increased marrow production of RBCs, granulocytes, and plts? | polycythemia vera
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| mutation implicated in polycythemia vera? | activating point mutations in tyr-kinase JAK2
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| difference between polycythemia vera and secondary absoulte polycythemia? | PCV = low EPO
secondary polycythemias = high EPO
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| pathogenesis of essential thrombocytosis? | same JAK2 point mutation as in PCV - receptor tyr-kinase activation makes progenitors thrombopoeitin-independent
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| what is erythromelalgia? | throbbing and burning of hands and feet caused by occlusion of small arterioles by plt aggregates
seen in ET and PCV
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| pathogenesis of primary myelofibrosis? | inappropriate release of PDGF and TGF-beta from neoplastic megakaryocytes (fibrogenic factors)
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| in what disease are large platelets and dacryocytes found? | myelofibrosis (dacryocytes = teardrop shaped RBCs damaged during birthing process in fibrotic marrow)
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| secondary disorder that can complicate the picture of MPDs? | hyperuricemia and gout (from high cell turnover rate)
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| infant develops cutaneous seborrheic-like eruption on chest, back, and scalp; also has hepatosplenomegaly, lymphadenopathy, pulmonary lesions. Dx? | Letterer-Siwe disease (Multifocal multisystem Langerhans cell histiocytosis)
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| What is the Hand-Schuller-Christian triad? | calvarial bone defects, diabetes insipidus, and exophthalmos
(multifocal unisystem Langerhans cell histiocytosis that involves the posterior pituitary stalk)
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| MCC of splenic infarctions? | emboli arising from the heart
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| MCC of thymic hypoplasia? | DiGeorge syndrome
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| MCC of thymic hyperplasia? | myasthenia gravis (also seen in other AI dz: Graves, SLE, scleroderma, RA)
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| difference between invasive thymoma and thymic carcinoma? | invasive thymoma is cytologically benign but locally invasive
thymic carcinoma is invasive, somtimes accompanied by metastases, usually squamous cell carcinoma
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