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WBC Path
LECOM WBC Path
| Question | Answer |
|---|---|
| Tennis rackets, Seborrheic eruptions, otitis media and mastoiditis | Langerhans cell histiocytosis |
| IgM Spike, hepatosplenmegaly and lacking hypercalcemia | lymphoplasmacytic lymphoma |
| Leukocytosis, immature myeloid cells in blood, increased leukocyte ALK PHOS | leukemoid reaction |
| Leukocytosis, immature myeloid cells in blood, low leukocyte ALK PHOS | CML |
| t(8;14), illeocecal mass, med. Leuks w/ coarse chromatin, high mitotic, starry sky | sporadic Burkitts |
| no HSM; Node/extranodal/tonsils large lymphoid cells large nuclei, CD 19, 20, 10 and BCL6 | DLCBL |
| High RBC(marked), platelets, & WBC; low erythropoietin | Polycythemia Vera |
| Polycythemia Vera “burns out” to | myelofibrosis |
| Polycythemia Ver ‘blast out” to | AML |
| Ringed sideroblasts, mafaloblasts, abnormal megakaryocytes, myeloblasts in marrow | myelodysplasia |
| Myelodysplasia with 5q deletion is caused by | post-therapy |
| High platelets &WBC, no marrow fibrosis, no polycythemia;large platelets | Essential Thrombocytosis |
| Virus associated w/ endemic Burkits lymphoma | EBV |
| Mass lesion in stomach, thyroid, or salivary, CD19/20+, CD3-, better after treatment | MALToma |
| <2yo, skin&pulmonary lesions, osteolytic lesion;Birbek granules | Letterer-Siwe Disease (Langerhans) |
| Typical cytoplasmic PAS + finding in AML | Auer Rod |
| anemia, thrombo/neutrocytopenia, marrow: delicate chromatin, nucleoli, az.granules | AML |
| Adult, cutaneous rash/lesions, numb, LA&HSM, anemic, lymphocytosis “clover leaf” cells | Adult TCL |
| Adult T-Cell Lymphoma/ Leukemia associated virus / countries | HTLV-1, Japan, W. Africa, Caribean |
| T or B Cell: CD1 | T-Cell |
| T or B Cell: CD3 | T-Cell |
| T or B Cell: CD4 | T-Cell |
| T or B Cell: CD5 | T-Cell |
| T or B Cell: CD8 | T-Cell |
| T or B Cell: CD10 | B-Cell |
| T or B Cell: CD19 | B-Cell |
| T or B Cell: CD20 | B-Cell |
| T or B Cell: CD21 | B-Cell |
| T or B Cell: CD23 | B-Cell |
| T or B Cell: CD79a | B-Cell |
| Lymphoid neoplasms mostly B or T cell? | B cell, 85-90% |
| BALL or TALL more common? | BALL 85% |
| Most common cancer of children? | ALL |
| ALL more common in what race? | hispanics |
| B-ALL age peak | 3yo |
| T-ALL age peak | “adolescence” |
| Mass present in 50-70% of T-ALLs | mediastinal thymic mass |
| ALL vs AML. ALL: ____chromatin, ______cytoplasm lacking ______ | difuse ; smaller amount; granules |
| ALL stains vs AML: myeloperoxidase ____ and cytoplasm PAS____ | myeloperoxidase negative; PAS + |
| Positive stain in 95% of both B-and T- ALL | TdT |
| TdT + and CD19+, CD10+ most likely Dx | B-ALL |
| Poor prognosis for ALL. Age? Genetics? | Under 2yo; peripheral blast >100k; t(9;22) Philadelphia chrm |
| Favorable prognosis for ALL. Age? Genetics? | 2-10yo; low WBC count; hyperploidy; trisomy 4,7,10; t(12;21) |
| Hyperdiploidy only seen in which? T-ALL or B-ALL? | B-ALL |
| GOF NOTCH1 mutation in T-ALL or B-ALL? | T-ALL |
| Philidelphia Chromosome # and fusion? | t(9;22); activated BRC-ABL gene |
| Most Common Cause of agranulocytosis | drug toxicity. |
| Common clinical manifestation of agranulocytosis | ulcerating, necrotizing lesions of the oropharyngeal mucosa |
| agranulocytosis predisposes to these deep fungal infections | Candida and Aprergillus |
| Below ___/mm3 neutrophils, serious Infx are likely | 500 |
| This IL stimulates mainly eosinophils | IL-5 |
| This GF induces neutrophilia | G-CSF |
| In sepsis or sever inflammatory reactions, leukocytosis is usually accompanied by these morphologic changes in neutrophils | toxic granulations, Dohle Bodies, and cytoplasmic vacuoles |
| Sky blue cytoplasmic puddles of dilated ER | Dohle bodies |
| Coarse, markedly darker abnormal azurophilic granules | toxic granules |
| Causes of neutrophilia | Acute bacterial Infx, usually pyogenic; MI or burns |
| Causes of eosinophilia | allergic disorders; parasite Infx; drug reactions; collagen vascular disorders |
| Causes of basophilia | rare; CML |
| Causes of monocytosis | chronic INFx; rickettsiosis, malaria; SLE; IBD |
| Causes of lymphosytosis | Viral Infx; pertussis; w/ monocytosis in chronic immunological stimulation (Tb) |
| t(9,22) is associated with what disease(s)? | ALL, AML, CML |
| what inherited genetic factors predispose to WBC neoplasias? | Bloom syndrome, Fanconi anemia, ataxia telangiectasia = Acute leukemias |
| Inherited genetic condition predisposing to childhood leukemias | +21, NF-1 |
| Which viruses predispose to WBC neoplasias? | HTLV-1, EBV, KSHV |
| Virus associated with adult T cell leukemia/lymphoma | HTLV-1 |
| Virus associated with Burkitt lymphoma, Hodgkin lymphoma, other B cell lymphomas in setting of T-cell immunodeficiency and NK-cell lymphomas | EBV |
| Virus associated with Kaposi sarcoma | KSHV |
| what environmental agent is associated with gastric B-cell lymphomas? | H. pylori infection |
| chronic immune stimulation in what disease is associated with intestinal T-cell lymphomas? | gluten-sensitive enteropathy |
| which plasma cell neoplasm often presents as pain d/t pathologic fractures? | multiple myeloma |
| 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) |
| MCC of cancer in children? | ALL |
| which subset of ALL presents as childhood leukemia? | B-ALL |
| which lymphoid neoplasm presents in adolescent males as thymic lymphomas (mediastinal mass)? | T-ALL |
| lymphoblasts express CD19 and karyotype shows hyperdiploidy and t(12,21)? | B ALL |
| lymphoblasts often express CD1a and gain-of-fxn mutations in NOTCH1? | T ALL |
| which WBC neoplasm causes compression of large vessels and airways in the mediastinum? | T-ALL |
| which drug is used in t(9,22)+ ALLs and inhibits BCR-ABL kinase? | Imatinib (Gleevec) |
| ALL responsiveness to aggressive chemo (including CNS prophylaxis)? | CR in 90% of children, 2/3 cured |
| MCC of leukemia of adults in Western world? | CLL |
| difference between CLL and SLL? | degree of peripheral blood lymphocytosis |
| WBC neoplasm in which abnml cells gather in LNs in proliferation centers; PBS contains smudge cells? | CLL/SLL |
| Neoplastic cells are positive for CD19, CD20, CD23, and CD5; deletions in 13q, 11q, 17p, trisomy 12q? | CLL/SLL |
| 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) |
| tumor that arises from germinal center B cells and is strongly assoc'd w/ translocations involving BCL2 | |
| t(14,18)]? | Follicular lymphoma |
| LN findings in follicular lymphoma? | centrocytes - small, cleaved cells centroblasts - larger cells w/ open chromatin, several nucleoli, modest amts of cytoplasm |
| 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 |
| MCC of NHL in United States? | follicular lymphoma |
| response of follicular lymphomas to chemo/rad? | survival is not improved by aggressive therapy |
| 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 |
| neoplasm with medium sized lymphocytes, "starry-sky" appearance, c-MYC t(8,14)? | Burkitt lymphoma |
| B-cell neoplasm assoc'd with EBV, presents in maxilla or mandible, involves kidneys, ovaries, adrenal glands? | African (endemic) Burkitt lymphoma |
| B-cell neoplasm with high incidence in AIDS, presents as mass involving ileocecum or peritoneum? | American (sporadic) Burkitt lymphoma |
| 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) |
| 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 |
| Disorder in which protein casts in DCT and collecting ducts are surrounded by multinucleated giant cells? | myeloma kidney |
| MCC of death in multiple myeloma? | infection (inc susceptibility d/t dec production of serum Ig's) |
| most common plasma cell dyscrasia? | monoclonal gammopathy of uncertain significance (MGUS) |
| MCC of elevated serum M protein level (< 3gm/dL) in asymptomatic older patient? | MGUS |
| B-cell neoplasm that infiltrates many organs, has M spike (IgM), but no hypercalcemia, and no lytic bone lesions present? | Lymphoplasmacytic lymphoma |
| what are Russell bodies and Dutcher bodies? | Russell bodies - cytoplasmic immunoglobulin Dutcher bodies - intranuclear immunoglobulin |
| characteristic of lymphoplasmacytic lymphoma | hyperviscosity syndrome |
| 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) |
| 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 |
| B-cell tumor that commonly occurs at sites of chronic immune or inflammatory reactions? | marginal zone lymphomas (MALTomas) |
| 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 |
| 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) |
| tumor of CD4+ T-cells that causes generalized pruritic erythematous rash (w/o hypercalcemia); abnml cells have cerebriform nuclei? | Mycosis fungoides/Sezary syndrome |
| 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 |
| rare neoplasm of CD8 T-cells, lymphocytes have abundant cytoplasm with coarse azurophilic granules? | Large granular lymphocytic leukemia |
| neoplasm presenting as descructive midline mass involving nasopharynx, skin, or testes; highly associated with EBV? | extranodal NK/T-cell lymphoma |
| 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 |
| neoplastic cell of this disease has "owl eye" bilobed nucleus w/ central nucleoli surrounded by clear space | Hodgkin lymphoma (Reed-Sternberg cells) |
| 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 | |
| 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 |
| 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 |
| type of HL with few lymphocytes, many RS cells? | lymphocyte depleted type |
| stereotyped progression of spread of HL? | nodal dz --> splenic dz --> hepatic dz --> marrow and other tissues |
| 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 |
| population at risk for AML? | adults |
| dx of AML is based on what? | >= 20% myeloid blasts in bone marrow |
| many myeloblasts on PBS, auer rods, peroxidase+ azurophilic cytoplasmic granules - Dx? | AML |
| auer rods are especially pronounced in what neoplasia (specific translocation)? | t(15,17) - acute promyelocytic leukemia |
| treatment of M3 subtype AML? | all-trans retinoic acid (ATRA) - induces differentiation of myeloblasts |
| 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) |
| 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) |
| disorder associated with t(8;14)? | burkitt lymphoma (c-myc activation) |
| disorder associated with t(14;18)? | follicular lymphomas (bcl-2 activation) |
| disorder associated with t(15;17)? | M3 type AML |
| disorder associated with t(11;22)? | Ewing's sarcoma |
| disorder associated with t(11;14)? | Mantle cell lymphoma |
| most common presentation of AML? | sxs related to anemia, neutropenia, TCpenia (fatigue, fever, spontaneous mucosal and cutaneous bleeding) |
| what factors contribute to bleeding diathesis in AML? | thrombocytopenia procoagulants and fibrinolytic factors released by leukemic cells (esp in t |
| 15;17]) | |
| which types of AML are high-risk? | those that follow MDS or genotoxic therapy or that occur in elderly |
| primary and t-MDS are associated with what karyotypic abnormalities? | monosomy 5 and 7; del 5q, 7q, 20q; trisomy 8 |
| most common finding in MDS? | dysplastic differentiation affecting all three myeloid lineages (erythroid, granulocytic and megakaryocytic) |
| 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 |
| what are pseudo-Pelger-Huet cells? | neutrophils with only two lobes (seen in MDS) |
| primary MDS affects what age group? | > 60 y |
| what are the chronic myeloproliferative disorders? | - CML - polycythemia vera - essential thrombocytosis - myelofibrosis w/ myleoid metaplasia |
| 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 |
| 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 |
| what lab finding helps differentiate between CML and other leukemoid reactions? | absence of leukocyte alk phos in CML |
| cell counts in CML - RBCs, WBCs, Plts? | dec RBCs inc WBCs inc Plts |
| pharm tx of CML? | Imatinib (anti-bcr-abl antibody) |
| disease characterized by increased marrow production of RBCs, granulocytes, and plts? | polycythemia vera |
| mutation implicated in polycythemia vera? | activating point mutations in tyr-kinase JAK2 |
| difference between polycythemia vera and secondary absoulte polycythemia? | PCV = low EPO secondary polycythemias = high EPO |
| pathogenesis of essential thrombocytosis? | same JAK2 point mutation as in PCV - receptor tyr-kinase activation makes progenitors thrombopoeitin-independent |
| what is erythromelalgia? | throbbing and burning of hands and feet caused by occlusion of small arterioles by plt aggregates seen in ET and PCV |
| pathogenesis of primary myelofibrosis? | inappropriate release of PDGF and TGF-beta from neoplastic megakaryocytes (fibrogenic factors) |
| in what disease are large platelets and dacryocytes found? | myelofibrosis (dacryocytes = teardrop shaped RBCs damaged during birthing process in fibrotic marrow) |
| secondary disorder that can complicate the picture of MPDs? | hyperuricemia and gout (from high cell turnover rate) |
| 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) |
| 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) |
| MCC of splenic infarctions? | emboli arising from the heart |
| MCC of thymic hypoplasia? | DiGeorge syndrome |
| MCC of thymic hyperplasia? | myasthenia gravis (also seen in other AI dz: Graves, SLE, scleroderma, RA) |
| 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 |
Created by:
csheck
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