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79 Cards in this Set
- Front
- Back
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WALDENSTROM MACROGOBULEMIA
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neoplastic B cells differentiate to IgM-producing cells (IgM spike)
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LEUKEMOID REACTION
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exagerrated response to infection, high leukocyte alkalaine phosphatase score
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CHRONIC MYELOID LEUKEMIA
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marked leukocytosis and immature myeloid cells, low leukocyte alkaline phosphatase score, philadelphia chromosome t(9;22); high WBC count
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HAIRY CELL LEUKEMIA
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peripheral blood leukocytes that mark w/tartate-resistance acid phosphatase; cells infiltrate the spleen and marrow; pancytopenia results from poor production of hematopoietic cells in the marrow and sequestration of the mature cells in the spleen; hairy projections from neoplastic leukocytes int he peripheral blood smeer and coexpression of B cell (CD19, CD20) and monocyte (CD11c) markers
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BURKITT LYMPHOMA
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african children, translocations of the MYC gene on chromosome 8, EBV, extranodal; B-cell lymphoma that typically appears in the maxilla or mandible of the jaw
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DIFFUSE LARGE B-CELL LYMPHOMA
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localized disease w/extranodal involvement, particularly of the Waldeyer ring, B Cells (CD19+)
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SMALL LYMPHOCYTIC LYMPHOMA
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B-cell neoplasm, widespread lymphadenopathy, hepatosplenomegaly, lymphocytosis
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HODGKIN LYMPHOMA
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Reed-Sternberg cells; involves lymph nodes in the middle or posterior mediastinum; older men; CD15, CD30; pain associated w/alcohol consumption is a paraneoplastic phenomenon peculiar to HL; RS cells are derived from B cells; within a given tumor, all RS cells have clonal (identical) immunoglobulin gene rearrangements; most contain EBV genome; lymphocyte depletion variant typically presents with advanced disease and EBV
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CHRONIC LYMPHADENITIS
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lymph node has many cell types - macrophages, lymphocytes, and plasma cells
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POLYCYTHEMIA VERA
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myeloproliferative disorder characterized by an increased RBC mass (blood volume) w/increased hematocrit concentrations > 60%; thrombocytosis, granulocytosis, low EPO, bleeding; some px "burn out" to myelofibrosis, some px "blast out" into acute myelogenous leukemia, some px develop chronic myelogenous leukemia; undetectable EPO in the face of polycythemia is characteristic of this disease; untreated, these patients die of episodes of bleeding or thrombosis--both related to disordered platelet function and the hemodynamic effects on distended blood vessels; treatment by phlebotomy reduces the hematocrit--with this treatment, the disease in 15-20% of px characteristically transforms into myelofibrosis w/myeloid metaplasia
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MYELODYSPLASIA
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cellular marrow in which there are maturation defects in multiple lineages, ringed sideroblasts, megaloblasts, abnormal megakaryocytes, and myeloblasts in the marrow, pancytopenia, susceptible to infections, clonal stem cell disorders that develop de novo or after chemotherapy w/alkylating agents, chromosomal deletion 5q is a marker of post-therapy myelodysplasia. essential thrombocythemia, CML, myelofibrosis, polycythemia vera; JAK2 mutation sare common
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ESSENTIAL THROMBOCYTHEMIA
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myeloproliferative disorder, myeloid stem cell, dominant cell type affected is megakaryocyte, thrombocytosis, increased platelet count, bone marrow: inc. megakaryotyctes with no evidence of leukemia, throbbing, buring pain in the extremiteis caused by platelet aggregates that occlude small arterioles; thrombotic or hemorrhagic crises
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HIV INFECTION
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risk factor for NH lymphoma
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MULTIPLE MYELOMA
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mass lesions of plasma cells in bone that leads to lysis and pain; "punched-out" lytic lesions (mediated by RANKL) in skull radiograph produced by expanding masses of plasma cells; Ig genes in myeloma cells always show evidence of somatic hypermutation; complicated by AL amyloid, which can also lead to renal failure; infections w/encapsulated bacteria b/c of decreased production of IgG; IL-6 important growth factor for plasma cells and correlates with active disease and poor prognosis; often produces a monoclonal serum "spike" seen in serum or urine protein electrophoresis; hypercalcemia and increased serum alkaline phosphatase level; neoplastic cells are generally well-differentiated, w/features such as perinuclear hof, similar to normal plasma cells
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LETTERER-SIWE DISEASE
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form of Langerhans cell histiocytosis; Birbeck granules found in cytoplasm
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SEZARY CELLS
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peripheral T-cell lymphoma/leukemias which often involve the skin
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ACUTE MYELOGENOUS LEUKEMIA
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young to middle-aged adults; peroxidase-positive myeloblasts and phenotypic features of myeloid cells; infiltrates the marrow and reduces normal hematopoiesis to account for anemia and marked thrombocytopenia; initial presentation may be acute
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T-CELL LEUKEMIA/LYMPHOMA VIRUS TYPE 1
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related to HIV-1, causes adult T-cell leukemia/lymphoma
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CHRONIC LYMPHOCYTIC LEUKEMIA
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older adults; patients have many small, round circulating mature B lymphocytes w/scan cytoplasm seen in the peripheral blood smear; clonal B-cell neoplasm in which immunoglobulin genes are rearranged, and T-cell receptor genes are in germline configuration; cells express the CD5 marker and the pan B cell markers CD19 and CD20; most patients have a disease course of 4 to 6 years before death, and symptoms appear as the leukemic cells begin to fillt he marrow; in some patients, the same small lymphocytes appear in tissues; the condition is then known as SMALL LYMPHOCYTIC LYMPHOMA
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t(8;14)
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BURKITT LYMPHOMA
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t(9;22)
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CML; philadelphia chromosome; BCR-ABL rearrangements, tyrosine kinase activated via this fusion gene is the target of current therapy for CML
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t(14;18)
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FOLLICULAR LYMPHOMAS (b-cell tumors); BCL2 gene
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t(11;14)
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MANTLE CELL LYMPHOMA; activates the cyclin DA (BCL1) gene
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t(15;17)
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ACUTE PROMYELOCYTIC LEUKEMIA
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NODULAR SCLEROSIS
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HL, young adults (women); bands of fibrosis; involvement of one group of lymph nodes is stage I; mediastinal involvement is common (stage I/II); NOT THYMUS; HL = Reed-sterberg cells present w/fibrous bands intersecting the lymphoid cells
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ACUTE LYMPHADENITIS
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painful and acute enlarged nodes is a reactive condition and not a neoplastic process, such as lymphoma or a leukemia
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TOXOPLASMOSIS
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produces a pattern of follicular hyperplasia
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GALACTOSEMIA
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splenomegaly, inborn error of metabolism, liver disease and fibrosis
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GAUCHER DISEASE
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splenomegaly w/o significant immunodeficiency
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ACUTE LYMPHOBLASTIC LEUKEMIA (precursor-B cell type)
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Rapid expansion of marrow caused by proliferation of blasts can lead to bone pain and tenderness; anemia, thrombocytopenia, and the presence of blasts in the peripheral blood and bone marrow; anemia and thrombocytopenia result from suppression of normal hematopoiesis by the leukemic clone in the marrow; phenotype of CD19+, CD3-, and sIg- is typical of pre-B cells
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TdT
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marker of early T cell-type and B cell-type lymphoid cells
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THYMOMA
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rare neoplasms that can be benign or malignant; 1/3 produce myasthenia gravis as an initial presentation are malignant; benign thymomas have a mixed population of lymphocytes and epithelial cells and are circumscribed; malignant thymomas are invasive and have atypical cells
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LYMPHOBLASTIC LYMPHOMA (T cell variety)
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thymus in children, no epithelial component; this lesion is within the spectrum of ALL; most cases of ALL with lymphomatous presentation are of the pre-T cell type; this fact is supported by the expression of the T-cell markers CD2 and CD7; look for young age and mediastinal location;
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ABSCESS
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granulation tissue at edge w/a mixture of inflammatory cell types, but not atypical cells
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LYMPHOID NEOPLASM
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derived from a single transformed cell and are monoclonal; 80/85% is B-cell neoplasm; can be shown by staining for light chains b/c mixture of B cells expressing kappa and lambda light chains; some have a follicular pattern
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CD30
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activated T and B cells
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MANTLE CELL LYMPHOMA
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CD5+, CD10-, t(11;14)
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HTLV-1
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cause leukemia/ymphoma and demyelinating disease; neoplastic lymphoid cells can infiltrate many organs;
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CAT-SCRATCH DISEASE
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R. henselae infection results in lymphadenopathy with microscopic stellate necrosis; a form of self-limited infectious lymphadenitis that most often is seen in children, typically "upstream" of lymphatic drainage from the site of injury, so that the axillary and cervical lymph node regions are most often involved
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PLAGUE
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Y. pestis can produce acute lymphadenopathy w/microscopic hemorrhagic necrosis
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FOLLICULAR LYMPHOMA
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most common form of non-Hodgkin lymphoma among adults in the US; men/women equally affected; neoplastic B cells mimic a population of follicular center cells and produce a nodular or follicular pattern; nodal involvement is often generalized, but extranodal involvement is uncommon; t(14;18) causes overexpression of the BCL2 gene making cells more resistant to apoptosis; indolent tumors that continue to accumulate cells for 7 to 9 years
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GRANULOMATOUS INFECTION
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leading to leukocytosis w/lymphocytosis and monocytosis; neutrophilia w/acute bacterial infections, such as s. aureus infection
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ASPERGILLOSIS
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cause of pulmonary nodules; neutropenia is a significant risk factor; organisms often invade blood vessels, producing hemorrhagic lesions
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BARTONELLOSIS
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bacillary angiomatosis (involves skin)
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SINUS HISTIOCYTOSIS
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represents an immunological response to cancer antigens
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CD68
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macrophage (histiocyte) marker
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POLYCLONAL PROLIFERATION
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benign reactive process
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MONOCLONAL PROLIFERATION
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suggests neoplasm
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AUER RODS
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myeloblasts in AML
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INFECTIOUS MONONUCLEOSIS
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large, "atypical" lymphocytes w/abundant, pale blue cytoplasm (seems to be indented by surrounding RBCs) and a large nucleus w/fine chromatin; caused by EBV and transmitted by close personal contact; multiple clones of B cells are infected with EBV; causes polyclonal B cell expnasion; B cells secrete antibodies w/several specificities, including antibodies that cross-react w/sheep RBCs; these heterophil antibodies produce a positive Monospot test result; atypical lymphocytes are CD8+ T cells that are activated by EBV-infected B cells; no increase in basophils, eosinophils, or monocytes
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"ALEUKEMIC" LEUKEMIA
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peripheral blood count of leukocytes is not high, but the leukemic blasts fillt he marrow; blasts show features of monoblasts b/c they are peroxidase negative and nonspecific esterase positive
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M5 AML
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characterized by high incidence of tissue infiltration and organomegaly
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ACUTE MEGAKARYOTCYTIC LEUKEMIA
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rare, typically accompanied by myelofibrosis, and the blasts react w/platelet-specific antibodies
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M3 AML
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promyelocytic leukemia, many promyelocytes filled with azurophilic granules, making them strongly peroxidase positive
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ERYTHROLEUKEMIA
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rare and is accompanied by dysplastic erythroid precursors
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GOOD PROGNOSIS FOR ALL
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early precursor-B celltype, hyperdiploidy, patient age b/w 7 and 10 yrs, chromosomal trisomy, and t(12;21)
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POOR PROGNOSIS FOR ALL
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T cell phenotype, patient age younger than 2 yrs, WBC count >100,000, presence of t(9;22), and presentation in adolescence and adulthood
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T-CELL ALL
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adolescents, mediastinal mass arises in thymus and lymphoid infiltrates appear in tissues of the mononuclear phagocyte system
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T-CELL LYMPHOMA
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skin involvement and presence of lymphocytes w/complex cerebriform nuclei in the skin and blood; malignancies of CD4+ and CD3+ T cells that may produce a tumor-like infiltration of the skin (mycosis fungoides) or a leukemic picture without tumefaction in the skin (Sezary syndrome); indolent tumors, and patients have a median survival of 8 to 9 years
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CD3-, CD56+
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phenotype: NK cells
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CD19+, sIg+
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phenotype: B cells
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CD33+, CD13+
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phenotype: monocytes/granulocytes
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CD19+, CD5+
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phenotype: neoplastic B cells in chronic lymphocytic leukemia
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MYELOFIBROSIS WITH MYELOID METAPLASIA
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also a stem cell disorder, in which neoplastic megakaryotcytes secrete fibrogenic factors leading to marrow fibrosis; the neoplastic clone then shifts to the spleen, where it shows trilineage hematopoietic proliferation (extramedullary hematopoiesis), in which megakaryotcytes are prominent; the marrow fibrosis and the extramedullary hematopoiesis in the spleen fail to regulate orderly release of leukocytes into the blood; the peripheral blood has immature RBC and WBC precursors (leukoerythroblastic picture)
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TEARDROP RBCs
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misshapen RBCs that are seen when marrow undergoes fibrosis
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MARROW INJURY W/O SPLENOMEGALY
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marrow injury as a result of metastatic tumors and irradiation
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CAUSES OF SPLENOMEGALY
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autoimmune disorders (SLE); infectious mononucleosis (antigenic stimulation of T cells); parasitic infections (malaria); primary and reactive (secondary) amyloidosis; lysosomal storage diseases (Gaucher's, Niemann-Pick); acute and chronic leukemias; hereditary spherocytosis; portal hypertension in cirrhosis; chronic myeloproliferative diseases (polycythemia vera, myelofibrosis and myeloid metaplasia, essential thrombocythemia)
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DIFFUSE LARGE-CELL LYMPHOMA OF B CELLS
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these tumors often involve extranodal sites, show large anaplastic lymphoid cells that involve the tissues diffusely, and contain BCL6 gene rearrangements; their clincial course is aggressive, and they become rapidly fatal if untreated; with intensive chemotherapy, however, 60 to 80% of patients achieve complete remission, and about 50% can be cured
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NOTCH1 GENE
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encodes for a transmembrane receptor required for T cell development, and more than half of pre-T cell tumors have activating point mutations
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LACUNAR CELLS + CD15+ REED-STERNBERG CELLS + FIBROUS BANDS
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NODULAR SCLEROSIS TYPE OF HODGKIN LYMPHOMA; more common in women
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LANGERHANS CELL HISTIOCYTOSES
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histiocytes with Birbeck granules
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ANAPLASTIC LARGE-CELL LYMPHOMA
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form of T-cell neoplasm most often appears in children and young adults; often extranodal and has a characteristic gene rearrangement on chromosome 2p23 that results in production of anaplastic lymphoma kinase (ALK) with tyrosine kinase activity
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REACTIVE CHANGES OF NEUTROPHILS
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toxic granulation, which are coarse and dark primary granules, and Dohle bodies, which are patches of dilated endoplasmic reticulum, represent reactive changes of neutrophils; these changes are most indicative of overwhelming inflammatory conditions, such as bacterial sepsis; route of infection in this case is probably intravenous drug use
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MONOCLONAL GAMMOPATHY OF UNCERTAIN SIGNIFICANCE (MGUS)
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characterized by the presence of an M protein "spike" in the absence of any associated disease of B cells; diagnosis of MGUS is made when the monoclonal spike is small (<3 g), and the patient has no Bence Jones proteinuria; MGUS can progress to multiple myeloma in about 20% of patients over 10 to 15 years
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HEAVY-CHAIN DISEASE
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rare condition that can be seen in chronic lymphocytic leukemia
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5q DELETION
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typical of myelodysplastic syndrome
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HELICOBACTER PYLORI
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can give rise to marginal zone lymphoma
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JAK2
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found in polycythemia vera and other myelo-proliferative diseases
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BCL6
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these gene rearrangements are typical of diffuse large B-cell lymphomas
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