Pathology Chapter 14 Vocab

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Acute leukemia

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characterized by a predominance of blasts and closely related cells in bone marrow and peripheral blood. Occurs most frequently in children – the most common malignanciy of pediatric age group. Also seen to have a secondary incidence peak after 60 years. Frequently associated with chromosomal translocation (9;22, Philadelphia). Includes ALL & AML classifications. Disease marked by infection, anemia, hemorrhage, and death within 6-12 months without intervention

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All-trans-retinoic acid

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The block in ApML patients caused by the (15;17) fusion and the presence of RARα-PML protein is overcome by pharmacologic doses of the vitamin A derivative all-trans-retinoic acid, causing neoplastic promyelocytes to differentiate into neutrophils. Like their normal counterparts, neutrophils derived from the neoplastic clone are short-lived and rapidly die, thus clearing the marrow and allowing resumption of normal hematopoiesis.

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ALL

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Acute Lymphoblastic Leukemia/Lymphoma. A group of neoplasms composed of immature, pre-B and pre-T lymphocytes referred to as lymphoblasts (while clinical overlap exists, ~85% are pre-B-cell and manifest as childhood acute “leukemias,” with bone marrow and peripheral blood involvement - ~15% due to pre-T-cell, which tend to present in adolescent males as “lymphomas,” often with thymic involvement). Immunophenotyping whether a malignant lymphoblasts is pre-B and pre-T, as they are morphologically indistinguishable, is neccessary. TdT will be + in 95% of cases

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AML

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Acute Myelogenous Leukemia. A neoplasm of immature myeloid cells that may cause signs and symptoms identical to ALL. Myeloblasts contain peroxides-positive granules as opposed to lymphoblasts which do not. TdT -, as myeloblasts do not possesses the specialized DNA polymerase necessary (lymphoblasts do in 95% of cases)

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Atypical lymphocyte

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morphological and physiological changes commonly occur in the lymphocytes of leukemia/lymphoma patients. Centrocytes – small lymphoid cells with condensed chromatin and irregular/cleaved nuclear outlines and centroblasts – largers cells with prominent nucleoli, are examples of atypical lymphocytes.

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Auer rod

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The cytoplasm often contains fine, azurophilic, peroxidase-positive granules. Distinctive red-staining peroxidase-positive structures called Auer rods, which represent abnormal azurophilic granules, are present in many cases and are particularly numerous in AML associated with the t(15;17) (acute promyelocytic leukemia) The diagnosis of AML is based on finding that myeloid blasts make up more than 20% of the cells in the marrow

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B cell CD markers

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Important for the classification of disease in leukemia/lymphoma patients. Primarily B-cell associated antigens include: CD10 (CALLA), 19 (not plasma cells), 20(after CD19, not plasma cells), 21(EBV receptor), 23 (activated mature B cells) and 79a (marrow pre and mature B cells).

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Balanced translocation

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A balanced translocation is a condition in which the correct number of chromosomes are present, but two pieces of chromosomal material have switched places.

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Bcl-2

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The hallmark of follicular lymphoma is a (14;18) translocation that juxtaposes the IgH locus on chromosome 14 and the BCL2 locus on chromosome 18. This translocation is seen in up to 90% of follicular lymphomas, and leads to overexpression of BCL2 protein. BCL2 is an antagonist of apoptotic cell death and appears to promote the survival of follicular lymphoma cells. Indeed, whereas reactive follicles contain numerous B cells undergoing apoptosis, neoplastic follicles are characteristically devoid of apoptotic cells.

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BCR/ABL

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Chronic myelogenous leukemia is distinguished from other chronic MPDs by the presence of a distinctive molecular abnormality, namely, a translocation involving the BCR gene on chromosome 9 and the ABL gene on chromosome 22. The resultant BCR-ABL fusion gene directs the synthesis of a 210-kDa fusion protein with tyrosine kinase activity. In more than 90% of CML cases, karyotyping reveals the so-called Philadelphia chromosome (Ph), which is created by a reciprocal (9;22)(q34;q11) translocation. In CML, multiple myeloid lineages, B lymphoid cells and possibly T lymphoid cells express the BCR-ABL fusion protein, indicating that the target of transformation is a pluripotent stem cell. CML is best differentiated from other chronic myeloproliferative disorders by detection of the BCR-ABL fusion gene, through either chromosomal analysis or PCR-based molecular tests.

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Bence-Jones protein

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Light chain overproduction by malignant B cell, seen in multiple myeloma.
Specifically, in Bence Jones proteinuria and cast nephropathy, the main cause of renal dysfunction is related to Bence Jones (light-chain) proteinuria, because renal failure correlates well with the presence and amount of such proteinuria and is uncommon in its absence. Two mechanisms appear to account for the renal toxicity of Bence Jones proteins. First, some light chains are directly toxic to epithelial cells; different light chains may have different nephrotoxic potential. Second, Bence Jones proteins combine with the urinary glycoprotein (Tamm-Horsfall protein) under acidic conditions to form large, histologically distinct tubular casts that obstruct the tubular lumina and also induce a characteristic inflammatory reaction around the casts (cast nephropathy).

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Birbeck granule

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The presence of Birbeck granules in the cytoplasm is characteristic for three disorders: Letterer-Siwe syndrome, Hand-Schuller-Christian disease, and eosinophilic granuloma. These three conditions are now considered different expressions of the same basic disorder of histiocytosis. The tumor cells in each are derived from dendritic cells and express HLA-DR, S-100, and CD1a. They have abundant, often vacuolated cytoplasm and vesicular nuclei containing linear grooves or folds in addition to the birbeck granules.

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Cat-scratch disease

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– Bartonella henselae organism causes cat-scratch disease in immunocompetent persons. It resides principally in the domestic cat. The gram-negative bacilli is cured by macrolide antibiotics.

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CD antigen

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Cluster of differentiation antigen. Used as a marker for cell-typing. Leukocytes express distinct assortments of molecules on their cell surfaces, many of which reflect either different stages of their lineage-specific differentiation or different states of activation or inactivation. Leukocyte cell surface molecules are routinely detected with anti-leukocyte monoclonal antibodies (mAbs). Using different combinations of mAbs, it is possible to chart the cell surface immunophenotypes of different leukocyte subpopulations, including the functionally distinct mature lymphocyte subpopulations of B-cells, helper T-cells (TH), cytotoxic T-cells (TC), and natural killer (NK) cells

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Chronic leukemia

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Characterized by proliferations of lymphoid or hematopoietic cells. Different from Acute Leukemia in that the cells involved are more mature. While longer in course, the clinical course is less devastating than AL. Subtypes involved include: Chronic Lymphocytic Leukemia, Hairy cell leukemia, and Chronic Myelogenous Leukemia.

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Cleaved cell

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Two principal cell types are observed in varying proportions in lymph nodes: (1) small cells with irregular or cleaved nuclear contours and scant cytoplasm, referred to as centrocytes (small cleaved cells), and (2) larger cells with open nuclear chromatin, several nucleoli, and modest amounts of cytoplasm, referred to as centroblasts. In most follicular lymphomas, small cleaved cells make up the majority of the cells.

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CLL

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Chronic lymphocytic leukemia. Characterized by a proliferation of neoplastic lymphoid cell (almost always B-cells) with infiltration of the bone marrow, peripheral blood, lymph nodes, spleen, liver, and other organs. Most often seen in patients over 60yrs

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Clonal evolution

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The clonal proliferative leukemia cells within bone marrow are particularly susceptible to mutation and divergence due to their ability to undergo hypermutation. In some cases, a translocation within a lineage of clones gives rise to a poorer prognosis (eg- Philadelphia chromosome 9;22).
In the case of lymphoid neoplasms, many of the oncogenic rearrangements stem from mistakes during the events that occur during antigen receptor gene expression. The normal immunoglobulin (Ig) and T-cell receptor gene diversity in B and T lymphocytes is produced by mechanisms relying on DNA breakage and rejoining. Athough the mechanism is still unsettled, it appears that "misdirected" somatic hypermutation also causes mutations in oncogenes that are implicated in B-cell transformation. The inherent genomic instability of germinal center B cells might explain why they are much more likely to give rise to lymphomas than are mature T cells, which have fixed, stable T-cell receptor genes.

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CML

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CML is distinguished from other chronic MPDs by the presence of a distinctive molecular abnormality, namely, a translocation involving the BCR gene on chromosome 9 and the ABL gene on chromosome 22. The resultant BCR-ABL fusion gene directs the synthesis of a 210-kDa fusion protein with tyrosine kinase activity. In CML, multiple myeloid lineages, B lymphoid cells and possibly T lymphoid cells express the BCR-ABL fusion protein, indicating that the target of transformation is a pluripotent stem cell.

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Cryoglobulin

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An abnormal blood protein that has the unusual properties of precipitating from the blood serum when it is chilled (hence the "cryo-") and redissolving when it is rewarmed. Cryoglobulins are caused by gamma globulins with a molecular weight of approximately 200,000.

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Cutaneous anergy

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absense of a normal immune response. Seen in most cases of Hodgkin Lymphoma; results from depressed cell-mediated immunity.

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cyclin D1

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Mantle cell lymphoma is associated with an (11;14) translocation involving the IgH locus on chromosome 14 and the cyclin D1 locus on chromosome 11.42 This translocation is detected in about 70% of cases by standard karyotyping and in an even higher fraction of tumors analyzed by fluorescence in situ hybridization. The t(11;14) leads to increased expression of cyclin D1, which promotes G1 to S phase progression during the cell cycle.

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Diffuse lymphoma

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lymphoma lacking a structural arrangement. The presence of an isolated large mass is typically seen in diffuse large B-cell lymphoma. Mantle-cell lymphoma exhibits the same diffusion of lymphocytes. Cytogenetic and gene expression profiling studies indicate that diffuse large B-cell lymphomas are heterogeneous in terms of their pathogenesis. One frequent pathogenic event in these tumors is dysregulation of BCL6, a DNA-binding zinc-finger transcriptional regulator that is required for the formation of normal germinal centers.

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endemic

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referring to one of the three different types of Burkitt Lymphoma (African/endemic, sporadic/nonendemic, and a subset of aggressive lymphomas occurring in individuals infected with HV). Essentially all endemic tumors are latently infected with EBV.

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Birbeck granules (HX Bodies)

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Cytoplasmic structures seen under electron microscopy in Langerhan’s cell histiocytosis
Have periodicity and dilated terminal end – look like a tennis racquet
Prominent infiltrate of reactive cells, including plasma cells, macrophages and eosinophils, is mixed with neoplastic histiocytes

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Eosinophilic granuloma (unifocal Langerhans cell histiocytosis)

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Pattern of Langerhan Cell histiocytosis (“Histiocytosis X”) - a clonal proliferation of these antigen-presenting dendritic cells, normally found in many organs
Affects skeletal system as an erosive, expanding accumulation of Langerhans cells within calvarium, ribs, femure, skin, lungs, or stomach
Asymptomatic or painful, found in children or young adults, esp. male
May remit spontaneously or be cured by excision or radiation

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Epstein-Barr virus (EBV) (Infectious Mononucleosis)

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Herpesvirus that causes benign, self-limited lymphoproliferative disease
Causes fever, lymphadenopathy, splenomegaly, sore throat, activated T-lymphocytes
Implicated in Burkitt lymphoma, Hodgkins Disease, many diffuse large B-cell lymphomas in T-cell immunodeficiency, and angiocentric lymphoma

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extramedullary hematopoiesis

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hematopoiesis in locations other than bone marrow, often seen in leukemia

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Extranodal lymphoma (often called a MALToma)

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Originates at postgerminal center of memory B cells
Arises at extranodal sites in adults with chronic inflammation or autoimmune diseases (eg, Sjogren – salivary glands, Hashimoto – thyroid, H.Pylori gastritis – stomach)
Genotype – Trisomy 18, t(11;18), t(1;14)>> MALT1-IAP2 + BCL 10-IgH genes
Typically surrounds/invades small vessels>> ischemic necrosis
Has large azurophilic granules resembling NK cells
Express NK-cell markers, contain EBV episomes (indicates origins)

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FAB classification (see chart p. 693 of Robbins)

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Classification of Acute Myelogenous Leukemias (AML)
Classes M0-M7, based on marrow morphology

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Flower cell (or cloverleaf cell)

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Cells with multilobular nuclei characteristic of Adult T-Cell Leukemia/Lymphoma

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Follicular hyperplasia

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Pattern of chronic nonspecific lymphadenitis
Caused by inflammatory processes that activate B cells
Distinguished by prominence of large, round germinal centers containing:
Dark zone: contains blastlike B cells (centroblasts)
Light zone: B cells with irregular nuclear contours (centrocytes)
Distinct from follicular lymphoma by:
Preservation of lymph node architecture (T-cell zones, sinusoids)
Variation in shape/size of lymphoid nodules
Frequent mitotic figures, phagocytic macrophages, light/dark zones

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Germinal center / secondary follicle

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a part of the lymph node in which lymphocyte proliferation and maturation takes place and where T-cells are "educated" to recognize antigens

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low-grade lymphoma

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tends to grow and spread slowly (“indolent”)

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Intermediate- or high-grade lymphoma

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Tends to grow and spread more quickly

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H-chain (heavy chain)

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H-chain and L-chains are involved in the production and coupling of normal plasma cells and are tightly balanced
Neoplastic plasma cells often synthesize excess L or H chains along with complete immunoglobulins

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Heavy chain disease

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common feature is synthesis and secretion of free H chain fragments
including chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma, and an unusual small bowel lymphoma that occurs in malnourished populations (so-called Mediterranean lymphoma)

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Histiocytosis X (Langerhans Cell Histiocytosis)

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Clonal proliferation of Langerhan’s cells (antigen presenting dendritic cells), normally found in many organs
Morphology – tumor cells have abundant, vacuolated cytoplasm with oval or indented nuclei
Express HLA-DR, Fc receptors and CD1a
E. microscopy – HX Bodies (Birbeck granules), have a periodicity and dilated terminal end - looks like a tennis racquet
Has prominent infiltrate of reactive cells – plasma cells, macrophages, eosinophils - mixed with neoplastic histiocytes

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Unifocal Langerhans cell histiocytosis (eosinophilic granuloma)

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Affects skeletal system as an erosive, expanding accumulation of Langerhans cells in calvarium, ribs, femur, skin, lungs, stomach
Asymptomatic or painful, in children or young adults, esp. male
May remit spontaneously or be cured by excision or radiation

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Multifocal Langerhans cell histiocytosis

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Affects children with fever, diffuse eruptions (part. scalp/ear canal), otitis media, mastoiditis, upper resp. tract inf., bone lesions, lymphadenopathy, hepatomegaly, splenomegaly
50%-post. pituitary stalk of hypothalamus involved>> diabetes insipidus (DI)
Hand-Schuller-Christian triad
Calvarial bone defects, DI, and exopthalmos

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Acute disseminated Langerhans cell histiocytosis (Letterer-Siwe disease)

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Aggressive systemic disorder, <2 yo,
Langerhans histiocytes infiltrate skin, spleen, liver, lung, marrow
Anemia, destructive bony lesions, rapidly fatal if untreated

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HIV-1

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lentivirus in a subgroup of retroviruses that are the cause of AIDS
leads to polyclonal B-cell activation and marked hyperplasia of germinal center B cells
HIV-infected individuals are at high risk for B-cell lymphomas derived from germinal center B cells, and most such tumors have oncogenic chromosomal translocations involving immunoglobulin loci

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HHV-8

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Kaposi sarcoma herpesvirus/human herpesvirus-8 (KSHV)
Uniquely associated with a Kaposi Sarcoma, an unusual type of B-cell lymphoma that presents as a malignant effusion, often with characteristic microscopic lesions of plump, spindle-shaped cells creating a slitlike vascular space filled with RBCs
Kaposi Sarcoma types (See Diseases of the Blood Vessels):
Classic(European)/Lymphadenopathic(African)/Transplant/AIDS

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HTLV-1

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associated with adult T-cell leukemia/lymphoma (ALL)

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Hypergranular promyelocytes

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Marrow cells of Acute promyelocytic leukemia

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Hyperviscosity syndrome

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Syndrome where blood is “thicker” than normal, leading to fatigue, weight loss and bleeding problems (eg, Waldenstrom macroglobulinemia)
A complication of:
Multiple Myeloma (due to excessive production and aggregation of M proteins (usually IgA))
Lymphoplasmacytic Lymphoma, (due to excess production of monoclonal IgM from a B cell neoplasm - Waldenstrom macroglobulinemia)

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Iatrogenic leukemia

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Leukemia caused by some other medical action, eg, the introduction of leukemia into CSF during lumbar puncture

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Ig gene rearrangement

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pathogenic rearrangements seen in lymphoid neoplasms caused by the inappropriate joining of these sites to sequences flanking proto-oncogenes

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Immunophenotype

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Classifying markers on tumor cells, eg. CD15, CD30 etc.

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Indolent

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Low-grade or slow growing, as in describing a neoplasm

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L-chain

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H-chain and L-chains are involved in the production and coupling of normal plasma cells and are tightly balanced
free L chains - Bence Jones proteins - are small enough to be rapidly excreted in urine
As a result, free L chains are detectable in blood only in setting of renal failure or very high levels of synthesis

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Primary or immunocyte-associated amyloidosis

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Results from a monoclonal proliferation of plasma cells secreting free L chains that are subsequently processed and deposited as amyloid
Some patients with primary amyloidosis have overt multiple myeloma, others have only a minor clonal population of plasma cells in the marrow

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L&H cell

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Subtype of Reed-Sternberg cell (distinctive for Hodgkin Lymphoma)
Lympho-histocytic variant cell with polypoid nuclei resembling popcorn kernels, inconspicuous nucleoli, and moderately abundant cytoplasm
Specific to lymphocyte predominance subtype
Reed-Sternberg cells – large, with either multiple nuclei or a single nucleus with multiple nuclear lobes, each with a large inclusion-like nucleolus about the size of a small lymphocyte

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Lacunar cell

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Subtype of Reed-Sternberg cell (distinctive for Hodgkin Lymphoma)
have delicate folded or multilobate nuclei surrounded by abundant pale cytoplasm that is often disrupted during the cutting of sections, leaving the nucleus sitting in an empty hole (the “lacune”)
Seen predominantly in nodular sclerosis subtype

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Left shift

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Rise in the number of more immature neutrophils in the blood associated with leukocystosis
Leukocytosis is a common feature of inflammatory reactions, especially those induced by bacterial infection
Occurs initially because of accelerated release of cells from the bone marrow postmitotic reserve pool

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Leukemia

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Lymphoid neoplasms presenting with widespread involvement of the bone marrow, usually accompanied by the presence of large numbers of tumor cells in the peripheral blood

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Leukemoid reaction

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Extreme elevations of leukocytes, typically because of proliferation of precursors in bone marrow and accelerated release of cells from bone marrow, induced by CSFs

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Leukocytosis

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Elevation in total white blood cell count, a common feature of inflammatory reactions, especially by bacterial infection

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Lymphadenitis

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Inflammation of the lymph nodes
infections and inflammatory stimuli elicit immune reactions within lymph nodes
two types: acute nonspecific and chronic nonspecific

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Lymphadenopathy

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Swelling of the lymph nodes

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Lymphoid follicle

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Localized concentrations of lymphocytes, commonly found in the walls of the alimentary canal, respiratory passages, and genitourinary tract
Are sharply defined, but not encapsulated

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Lineage restriction

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seen in adult stem cells that have a more restricted differentiation capacity

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Lymphoblast

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A lymphocyte that has enlarged following stimulation by an antigen, has the capacity to recognize the stimulating antigen, and is undergoing proliferation and differentiation either to an effector state in which it functions to eliminate the antigen or to a memory state in which it functions to recognize the future reappearance of the antigen

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Lymphoma

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Any of various (usually malignant) neoplasms of lymphatic and reticuloendothelial tissues that occur as circumscribed solid tumors and that are composed of cells that resemble lymphocytes, plasma cells, or histiocytes

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M-component

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A monoclonal immunogloblulin from proliferations of B-cell clones
It is so heavy (160,000kD) that it is restricted to the plasma and extracellular fluid and is excluded from the urine in the absence of glomerular damage

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Macroglobulin

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Hypersecreted monoclonal IgM seen in lymphoplasmacytic lymphoma
Amounts of IgM are enough to cause a hyperviscosity syndrome called Waldenström macroglobulinemia

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Mantle zone

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part of the lymph node where one finds B-cells surrounding an atrophic germinal center in mantle cell lymphoma
-Mantle cell lymphoma is associated with an (11;14) translocation involving the IgH locus on chromosome 14 and the cyclin D1 locus on chromosome 11

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Marginal zone

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These are a class of B-cell disorders that arise within the lymph nodes, spleen, or extranodal tissues.
-Examples include tumors arising in the salivary gland in Sjögren disease, the thyroid gland in Hashimoto thyroiditis, and the stomach in Helicobacter gastritis.

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Monoclonal gammopathy

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A disorder of B-cells in which one type of immunoglobulin (a clone) is produced
Associated with Multiple myeloma, Solitary myeloma, Waldenström macroglobulinemia, Heavy-chain disease, Primary or immunocyte-associated amyloidosis, and Monoclonal gammopathy of undetermined significance (MGUS)

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monoclonal/polyclonal

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describes B-cell disorders that produce either one type of immunoglobulin (monoclonal) or many types (polyclonal)

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Myeloblast

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a large mononuclear nongranular bone marrow cell that is a precursor of a myelocyte

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Myelocytic leukemia

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a disorder that results in decreased mature myelocytes, usually from exposure to ionizing radiation (think atomic bomb in WWII)

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Myelodysplasia

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Seen in Hodgkin’s lymphoma patients as a result of alkylating chemotherapeutic drugs and causes ringed sideroblasts, erythroid progenitors with iron-laden mitochondria, pseudo-Pelger-Hüet cells, neutrophils with only two nuclear lobes instead of the normal three to four, and, megakaryocytes with multiple nuclei instead of the normal single multilobated nucleus

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Myelofibrosis

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Results from collagen deposition by non-neoplastic fibroblasts
Suppresses bone marrow hematopoiesis, leading to peripheral blood cytopenias and extensive neoplastic extramedullary hematopoiesis in the spleen, liver, and lymph nodes
Can see teardrop-shaped cells

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Myeloid metaplasia

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-A syndrome characterized by anemia, splenomegaly, and the presence of nucleated red blood cells and immature granulocytes in the blood
-Accompanied by extramedullary hematopoiesis in the spleen and liver

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Myeloma

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a primary tumor of the bone marrow formed of any one of the bone marrow cells (as myelocytes or plasma cells) and usually involving several different bones at the same time

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Myeloma nephrosis

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Caused by accumulation of Bence Jones proteins in the Kidney. Associated w/ Multiple Myeloma

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Myelomonocytes

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– Phenotypic hybrids of myelocytes and monocytes seen in AMMoL (M4 AML)

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Myeloproliferative disorder

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Characterized by autonomous production or one or more hematopoietic elements in the bone marrow. The spleen and liver may be enlarged. Examples are Chronic Myelogenous Leukemia, Polycythemia Vera, Essential thrombocytosis, and Myelofibrosis with myeloid metaplasia

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Naïve B cell

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B cell that has not come in contact with its specific antigen.

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Nodular sclerosis

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Most common form of Hodgkin Disease. Characterized by the presence of lacunar cells (variant of Reed Sternberg cells) and Collagen bands that divide the lymph tissue into nodules

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Opportunistic infection

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A disease caused by an organism that is only pathogenic in an immunocompromised host.

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P.vera – Polycythemia vera

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A myeloproliferative disorder characterized by bone marrow hypercellularity (all lineages), Peripheral basophilia and neutrophilia, Giant platelets, Megakaryocytic fragments, and mild organomegaly. Late in its course it transforms to marrow fibrosis.
Symptoms usually related to high levels of RBCs

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Paracortex of lymph node

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T-cell region of the lymph node. Below the Cortex, where the germinal centers are found

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Peripheral cell origin

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mature cell neoplasms. Ex. Peripheral T-cell lymphoma – histologically and genetically heterogeneous tumors of mature t-cells. Usually presents in adults within lymph nodes. Aggressive course.

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Philadelphia chromosome

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9:22 chromosomal rearrangement. Associated w/ CML.

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Promyelocyte

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Myeloid precursor cell. First cell stage after myeloblast. First stage to contain granules (Primary granules).

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Precursor cell origin

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Referring to tumor origin. T and B cell precursor neoplasms are aggressive neoplasms of immature TdT+ cells
B-cell – ALL – usually presents w/ extensive marrow and peripheral blood involvement. Genetically heterogeneous. Most common in childhood.
T-cell – ALL – often presents as a mediastinal mass. Genetically heterogeneous. Most common in adolescent boys.

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Primary amyloidosis

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Also called B cell Dyscrasias - Most common type, composed of AL-type amyloid, can be seen in association w/ Multiple myeloma and monoclonal Ig. Majority of cases are not associated w/ b-cell neoplasm, but still have monoclonal Ig. Typically involves the heart, GI tract, peripheral nerves, skin, and tongue.

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prolymphocyte

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Lymphoid precursor cell. Stage that follows lymphoblast

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Reed-Sternberg cell

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Distinctive neoplastic giant cell that is the defining cell in Hodgkin Lymphoma. Has a single bi-lobed nucleus or multiple nuclei w/ large nucleolus in each lobe/nucleus.

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Retinoic acid receptor

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receptor for vitamin A. t(15:17) causes a mutation in the receptor that causes a halt in myelocytic development at the promyelocyte stage. This can be overcome with high doses of all-trans-retinoic-acid which causes the neoplastic cells to differentiate into neutrophils and subsequently die (death by differentiation).

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Sezary cell

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Seen in sezary syndrome or peripheral T-cell leukemia, a disease of neoplastic T-cells involving an exfoliative erythroderma. Cells have marked infolding of the nuclear membrane.

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smudge cell

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Small, round lymphocytes with scant cytoplasm that are frequent disturbed (smudged or broken). Seen in CLL.

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Sinus histiocytosis or reticular hyperplasia

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Lymphadenitis characterized by distension and prominence of the lymphatic sinusoids from hypertrophy of endothelial cells and inflammation of histiocytes. Often seen in areas that drain a cancer.

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Stage of lymphoma

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Involvement of a single lymph node region or extralymphatic organ or site.
Involvement of two or more lymph node regions on the same side of the diaphragm. May involve extralymphatic organ or tissue.
Involvement of lymph node regions on both sides of the diaphragm. May include the spleen or limited contiguous extralymphatic organ of site.
Multiple or disseminated foci or involvement of one or more extralymphatic organs or tissues with or without lymphatic involvement.

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Stellate abscess/ necrotizing granuloma

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a star-shaped necrotic area surrounded by histiocytes

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T cell CD markers

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CD1,3,4,5,8. Precursor cells are usually negative for CD4,8, but have CD7

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Terminal deoxytransferase (TdT)

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special DNA polymerase expressed only by pre-B and pre-T lymphocytes.

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What is Trisomy 12 associated with

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Chronic Lymphocytic Leukemia; associated w/ poor outcome

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Acute Myelogenous Leukemias WHO classification

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M0 – mimimally differentiated AML
M1 – AML without differentiation
M2 – AML with maturation
M3 – Acute Promyelocytic leukemia
M4 – Acute Myelomonocytic Leukemia
M5 – Acute Monocytic Leukemia
M6 – Acute erythroleukemia
M7 – Acute megakaryocytic leukemia

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WHO classification

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World Health Organization classification of Acute myelogenous leukemias and lymphoid neoplasms.

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Lymphoid Neoplasms

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Precursor B-cell and T-cell neoplasms
Peripheral B-cell neoplasms
Plasma cell neoplasms and related disorders
Peripheral T-cell and NK-cell neoplasms
Hodgkin lymphoma