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10.25 Hematology
8:30 by Dr. Jetly
226
Pathology
Professional
10/25/2011

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Term
What are the features of chronic nonspecific lymphadenitis?
Definition
follicular hyperplasia, paracortical hyperplasia, sinus histiocytosis
Term
Name the Non-hodgkin B cell lymphomas?
Definition
chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), mantle cell lymphoma, marginal zone lymphoma, follicular lymphoma, plasmacytoma/plasma cell myeloma, diffuse large B0cell lymphoma, burkitt lymphoma
Term
Name the T cell non hodgkin's lymphoma?
Definition
anaplastic large cell lymphoma, cutaneous T cell lymphoma, extranodal NK/T-cell lymphoma, peripheral T cell lymphoma (unclassified)
Term
Name the two Hodgkin's lymphomas.
Definition
nodular lymphocyte predominant hodgkin lymphoma; classic HL (nodular sclerosis, mixed cellularity, lymphocyte-rich, lymphocyte-depleted)
Term
What is the B cell area of the lymph node?
Definition
cortex
Term
What is the t cell area of the normal lymph node?
Definition
paracortex
Term
Where is the plasma cell area of a normal lymph node?
Definition
medulla
Term
Where in a normal lymph node are histiocytes found?
Definition
in the sinuses
Term
What is a typical lymph node size?
Definition
<1cm
Term
A fluctuant rubbery node is typical of...
Definition
suppurant (pus) nodes
Term
"shotty" nodes that feel like buckshot under the skin are typical of...
Definition
children with viral illnesses
Term
What is matted lymphadenopathy?
Definition
nodes feel connected and can move as a unit or be fixed in place. May be benign (TB, sarcoid) or malignant (lymphoma or mets)
Term
In what location does LAD have the highest risk of metastatic malignancy?
Definition
supraclavicular (90% for pts over 40, 25% in pts under 40)
Term
Where is cat scratch disease LAD usually located?
Definition
cervical or axillary
Term
What are the MC sites of nonspecific acute lymphadenitis?
Definition
cervical= oral cavity infections
inguinal= infections of extremities
Term
A generalized lymphadenopathy can be due to what kind of situation?
Definition
child with systemic viral infection
Term
What is the physical exam findings of nonspecific acute lymphadenitis?
Definition
nodes are tender, fluctuant, and the over lying skin is reddened
Term
What is the histological appearance of nonspecific acute lymphadenitis?
Definition
follicular hyperplasia with neutrophils around the follicles and within the sinuses
Term
What are the clinical characteristics of cat scratch lymphadenitis?
Definition
axillary or cervical lymphadenopathy, history of exposure to cats/scratches on arms
Term
What is the histology of cat scratch lymphadenitis?
Definition
stellate microabscesses, necrotizing granulomas
bartonella henselae (gram negative bacillus) seen on warthin-starry stain
Term
What types of diseases cause folllicular hyperplasia?
Definition
early HIV, RA, toxoplasma
Term
What are the features of follicular hyperplasia associated with crhonic nonspecific lymphadenitis?
Definition
preservation of LN architectures, polarized mantle zone, well defined germinal centers that vary in size and shape, and tingible body macrophages
Term
What causes paracortical hyperplasia?
Definition
immunologic reaction to drugs (dilantin), acute viral infection (IM), poast vaccination
Term
What are the characteristics of paracortical hyperplasia?
Definition
T-cell activation, T cells encroach and efface germinal centers, hypertrophy of sinusoidal and vascular endothelial cells
Term
What causes sinus histiocytosis?
Definition
lymphatic sinuses distended with histiocytes; seen in lymph nodes draining carcinoma (e.g. breast)
Term
What is a leukemia?
Definition
proliferation in the bone marrow and usually the peripheral blood. Can be acute (immature/blasts) or chronic (mature)
Term
What are the different types of leukemia?
Definition
granulocytic, monocytic, erythrocytic, lymphocytic
Term
What is a lymphoma?
Definition
proliferation of lymphoid cells in discrete tissue masses usually not seen in peripheral blood
Term
Are lymphomas malignant or benign?
Definition
all malignant but behavior can range from indolent to aggressive
Term
What percent of lymphoid neoplasms are B cell origin?
Definition
80-85%
Term
T/F All lymphoid neoplasms are monoclonal proliferations.
Definition
true, they are all derived from a single transformed cell
Term
T/F You need histologic examination of lymphoid neoplasms in order to diagnose them.
Definition
true
Term
How do you diagnose lymphomas?
Definition
microscopic recognition of the dominant cell type supplemented by immunologic and cytogenetic/molecular techniques
Term
How do you treat lymphomas/determine the prognosis?
Definition
based upon the dominnat cell type (and its inherent biological behavior), extend of spread (stage), and underlying health of the patient
Term
T/F Indolent lymphomas can further mutate and transform to aggressive types.
Definition
true
Term
What are the characteristics of hodgkin's lymphoma?
Definition
localized to a single axial group of nodes, oderly spread by contiguity, mesenteric nodes and waldeyer ring rarely involved, extranodal involvement uncommon
Term
What are the characteristics of nonhodgkin lymphoma?
Definition
more frequent involvement of multiple peripheral nodes, noncontiguous spread, waldeyer ring and mesenteric nodes commonly involved, extranodal involvement common
Term
Name some supplemental/ancillary techniques to diagnose lymphoma/leukemia?
Definition
flow cytometry immunophenotyping, immunohistochemistry on tissue sections, cytogenetics
Term
How do you perform flow cytometry/immunophenotyping?
Definition
cells flow through a smal quartz capillary tube at a constant velocity and are illuminated by 1 or more focused light sources. Forward scatter measures cross sectional area/size. Side scatter measures complexity/granularity
Term
How is B cell clonality determined via flow cytometry immunophenotyping?
Definition
light chain restriction (kappa versus lambda)
Term
What enzyme is used in immunohistochemistry?
Definition
immunoperoxidase based staining
Term
What are B-cell markers?
Definition
CD19, 20, 23, 79a and surface immunoglobulins
Term
What are t cell merkers?
Definition
CD1a, 2, 3, 4, 5, 7, 8
Term
What are NK markers?
Definition
CD 2, 7, 16 and 56
Term
What translocation is associated with mantle cell lymphoma?
Definition
t (11;14)
Term
What translocation is associated with follicular lymphoma?
Definition
t(14;18)
Term
What translocation is associated with burkitt lymphoma?
Definition
t(8:14)
Term
What translocation is associated with anaplastic large cell lymphoma?
Definition
t(2;5)
Term
Name some examples of small cell, mature B cell neoplasms?
Definition
chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), mantle cell lymphoma, follicular lymphoma, marginal zone lymphoma
Term
Name an example of a plasma cell mature b cell neoplasm?
Definition
plasmacytoma, plasma cell myeloma
Term
Name an example of a large cell mature b cell neoplasm.
Definition
diffuse large b cell lymphoma
Term
Name an example of a medium cell mature b cell neoplasm.
Definition
burkitt lymphoma
Term
WHat is a precursor b cell neoplasm?
Definition
precursor b lymphoblastic leukemia/lymphoma
Term
What is chronic lymphocytic leukemia/small lymphocytic lymphoma?
Definition
neoplasm of monomorphic small, round B lymphocytes in the peripheral blood, bone marrow and lymph nodes
Term
What's the differenct between chronic lymphocytic leukemia and small lymphocytic lymphoma?
Definition
SLL is restricted to tissue, non-leukemic; CLL invovles bone marrow and peripheral blood
Term
How many lymphocytes are in the peripheral blood when you have CLL?
Definition
>4,000 lymphocytes
Term
What is the most common leukemia in the western world?
Definition
crhonic lymphocytic luekemia
Term
What percent of NHL is SLL?
Definition
4%
Term
What is the age and gender preference of CLL/SLL?
Definition
most common >50 yoa (median is 60)
M:F::2:1
Term
What are the symptoms and prognosis of CLL and SLL?
Definition
frequently asymptomatic and median survival is 7 years
Term
What is the immunophenotype of CLL and SLL?
Definition
coexpresion of CD19 or CD20 (b cell marker) with CD5 (t cell marker). Positive CD23
Term
What is the appearance of mantle cell lymphoma cells?
Definition
resemble normal mantle layer cells: small, slightly irregular nuclei with a nodular architecture.
Term
What is the immunohistochemistry of mnatle cell lymphomas?
Definition
CD19+, CD20+, CD5+, Cyclin D1+
CD23-
Term
Describe the pathology behind the translocation of mantle cell lymphoma?
Definition
juxtaposes cyclin D1 gene on chromosome 11 next to immunoglobulin heavy chain gene on chrmosome 14. This causes over-expression of cyclin D1 a protein that promotes proliferation
Term
How do you treat/what is the prognosis of mantle cell lymphoma?
Definition
poor, tx with BMT
Term
What is the most common NHL in the US?
Definition
follicular lymphoma (45%)
Term
What is the age/gender preference of follicular lymphoma?
Definition
59 yoa; M:F::1:1.7
Term
How do follicular lymphomas present?
Definition
painless, generalized LDN
Term
What percent of follicular lymphomas invovle the bone marrow?
Definition
85% (paratrabecular, high stage)
Term
What is the prognosis of follicular lymphoma?
Definition
indolent course but incurable; 7-9 year life expectancy
Term
Do follicular lymphomas transform to aggressive lymphomas?
Definition
yes 30-50% do; frequently diffuse large B cell
Term
What is the histology of follicular lymphoma?
Definition
nodular growth pattern; neoplastic cells resemble normal germinal center B cells
Term
What is follicular lymphoma a tumor of?
Definition
centrocytes (small cleaved cells), and centroblasts (large cells with open chromatin and prominent multiple nucleoli
Term
What are the CD markers of follicular lymphoma?
Definition
CD19+, 20+, 10+ (CALLA), Bcl2+
CD5-
Term
Describe the translocation involved in follicular lymphoma?
Definition
t(14:18) leads to an over expression of Bcl2 which is an anti-apoptotic protein. FL stain positively for Bcl2 and reactive follicles are negative
Term
What is the cell origin of marginal zone lymphoma?
Definition
memory b cell origin
Term
What are the different types of marginal zone lymphomas?
Definition
may be nodal, extranodal (MALT); splenic
Term
Infections cause what types of marginal zone lymphomas?
Definition
gastric (malt), (helicobacter pylori)
Term
What kinds of autoimmune diseases are associated with  marginal zone lymphoma?
Definition
thyroid (hashimoto's), salivary glands (Sjorgren's)
Term
What is the prognosis/course of marginal zone lymphomas?
Definition
usually localized, indolent; can get generalized late in course of disease, may regress if inciting agent is eradicated
Term
What is the histology of marginal zone lymphomas?
Definition
small cells with variable mixture nuclear shapes (round, irregular, cleaved)
Term
What are the markers for marginal zone lymphoma?
Definition
CD19+, CD20+, CD5-, CD10-
Term
Small B cell lymphomas all have which CD markers in common?
Definition
CD19+, CD20+, CD3-
Term
What is a tumor that has CD19+, CD20+, CD3-, CD5+, CD23+, CD10-?
Definition
CLL/SLL
Term
What kind of tumor is CD19+, CD20+, CD3-, CD5+, CD23-, CD10-, Cyclin D1+?
Definition
mantle cell lymphoma
Term
What kind of tumor has CD19+, CD20+, CD3-, CD5-, CD23-, CD10+, Bcl2+?
Definition
follicular lymphoma
Term
What kind of tumor is CD19+, CD20+, CD3-, CD5-, CD23-, CD10-?
Definition
marginal zone lymphoma
Term
What is diffuse large b cell lymphoma?
Definition
fastr growing tumor composed of large B cells that presents as a rapidly enlarging mass at nodal or extranodal sites and can be rapidly fatal if untreated
Term
What is the age/gender preference for diffuse large B cell lymphoma?
Definition
male predominance; median age is 60 yrs
Term
What percent of NHLs are diffuse large b cell lyphomas?
Definition
20-40% (most common ?)
Term
What is the prognosis of diffuse large b cell lymphoma?
Definition
rapidly fatal if untreated. 60-80% remission with aggressive chemo. 50% potentially "cured"
Term
What percent of diffuse large b cell lymphomas have extranodal involvement?
Definition
40%
Term
AntiCD20 (rituximab) is used for what lymphoma/leukemia?
Definition
diffuse large b cell lymphoma
Term
HIV related diffuse large b cell lymphoma is usually...
Definition
EBV+
Term
T/F Burkitt lymphoma is an indolent neoplasm.
Definition
false, highly aggressive
Term
Burkitt lymphoma often presents in _______ sites.
Definition
extranodal
Term
What age/gender is common for burkitt lymphoma?
Definition
children/young adults; M:F::2-3:1
Term
What are the three different forms of burkitt lymphoma?
Definition
endemic, sporadic (non endemic), HIV related
Term
What are the characteristics of endemic burkitt's lymphoma?
Definition
africa, jaw, abdominal viscera, all EBV+
Term
What are the characteristics of sporadic (nonendemic) burkitt's lymphoma?
Definition
extranodal, abdominal mass, ileocecum, most EBV negative
Term
What are the characteristics of HIV-related burkitt's lymphoma?
Definition
usually eaerlier than large B cell lymphoma, most eBV+
Term
What is the histology of Burkitt lymphoma?
Definition
diffuse infiltrate of monomporphic medium sized B cells with basophilic cytoplasm and round nuclei with 2-5 nucleoli. Also has many interspersed histiocytes ("starry sky" pattern) with many mitotic figures and apoptotic bodies
Term
What are the markers for burkitt lymphoma?
Definition
CD10+, CD20+, Bcl6+, Bcl2-, TdT-
Term
What is the proliferaiton index of burkitt's lymphoma?
Definition
100% (Ki67 stain)
Term
Describe the pathology of the translocation behind burkitt's lymphoma.
Definition
juxtaposes c-myc (ch. 8) with IgH (cg. 14) cuasing over expression of cmyc which is a pro-proliferative/anti-apoptotic protein
Term
Plasma cell neoplasms account for what percent of all hematologic malignancies?
Definition
15%
Term
What are the different types of plasma cell neoplasms?
Definition
plasma cell myeloma (multiple myeloma), plasmacytoma, monoclonal immunoglobin deposition diseases (amyloidosis), heavy chain disease
Term
What is the most common lymphoid malignancy in blacks?
Definition
multiple myeloma
Term
What is the age/gender of Multiple myeloma?
Definition
68-70 yoa; M:F::1:1
Term
What is the prognosis of multiple myeloma?
Definition
usually incurable; median survival rate is 3 years
Term
What are the CD markers of multiple myeloma?
Definition
CD38+, CD79a+, CD138+, usually CD56+
Term
What parts of the skeleton are typically involved in MM?
Definition
vertebrae, ribs, skull, pelvis, femur, clavicle, and scapula
Term
What are the clinical features of MM?
Definition
C= hypercalcemia
R= renal failure; recurrent infections
A= anemia
B= bone lesions, "punched out", vertebral collapse, bone pain, pathologic fx
Term
T/F MM is usually associated with hepatosplenomegally and lymphoadenopathy?
Definition
false
Term
What is the #1 cause of death for MM patients?
Definition
infection
Term
What do you call proteins in the urine as a result of MM?
Definition
bence jones proteins
Term
Which plasmacytomas usually progreses to MM?
Definition
solitary plasmacytoma of bone
Term
Describe the features of plasmacytomas and give two examples of plasmacytomas?
Definition
solitary lesions of bone or soft tissue that are plasma cell tumors and can be controlled locally by radiation therapy 1) solitary plasmacytoma of bone 2)extraosseus plasmacytoma
Term
What are examples of precursor T cell neoplasms?
Definition
precursor T lymphoblastic leukemia/lymphoma; blastic NK cell lymphoma
Term
What percent of NHL are mature T cell neoplasms?
Definition
15%
Term
Which lymphoma is more common in asia?
Definition
mature T cell neoplasms
Term
What are the CD markers of mature T cell neoplasms?
Definition
CD2, 3, 5, 7, CD4>CD8
Term
What are the immunologic markers of T cell clonality?
Definition
there are none: the loss of normally expressed antigens implies T cell clonality. Loss of CD5 and CD7 is most common
Term
What are the two types of anaplastic large cell lymphomas?
Definition
ALK positive and ALK negative
Term
What is the age range of ALCL, ALK+?
Definition
3% of adult NHLs and 10-30% of childhood lymphomas
Term
Where are ALCL, ALK+ tumors?
Definition
both lymph nodes and extranodal sites
Term
What are the symptoms of ALCL, ALK+?
Definition
B symptoms; present with high stage disease
Term
What are the histological/immunological characteristics of ALCL, ALK+?
Definition
CD30+, ALK+, t(2;5)/NPM-ALK, loss of multiple pan-t cell antigens
Term
What is ALCL, ALK-?
Definition
provisional category in WHO 2008 for older age group 40-65 yoa. poorer prognosis
Term
Where aer ALCL ALK- cases?
Definition
less freqently in extranodal sites
Term
What does ALK stand for?
Definition
anaplastic lymphoma kinase
Term
What is the histological hallmark for ALCL?
Definition
horseshoe shapped ALCL hallmark cells
Term
What is the survival rate for ALK+ ALCL versus ALK- ALCL?
Definition
ALK+= 80% 5 year survival
ALK-= 40% 5 year survival
Term
What percent of patients with ALCL present with stage III to stage IV disease? What are the characteristics of stage III/IV?
Definition
70% (with peripheral and/or abdominal dz, often with marrow involvement, often with extranodal sites; often with B symptoms)
Term
What is teh translocation pathology of ALCL?
Definition
t(2;5) juxtaposes ALK on ch 2 and NPM on ch.5. ALk encodes a tyrosine kinase protein that is oncogenic
Term
What causes cutaneous T cell lymphoma?
Definition
CD3+, CD4+ helper T cells that home to the skin
Term
T/F Mycosis fungoides and sezary syndrome are different manifestations of a single neoplastic entity.
Definition
true
Term
What is mycosis fungoides?
Definition
early pathc lesion with plaque and tumor stages. Indolent course
Term
What are the characteristics of sezary syndrome?
Definition
generalized disease. Triad of erythroderma, generalized LAD, and sezary cells in lymph nodes, skin or peripheral blood
aggressive dz
Term
What histological feature is characteristic of mycosis fungoides?
Definition
pautrier microabscesses
Term
What neoplasm has cerebri looking lymphocytes?
Definition
sezary syndrome
Term
What part of the world is endemic for extranodal NK/T-cell lymphoma, nasal type?
Definition
asians (rare in western)
Term
What patient population is most common for extranodal NK/T-cell lymphoma, nasal type?
Definition
asians, immunosuppressed, post-transplants pts
Term
What are the physical symptoms of extranodal NK/T-cell lymphoma, nasal type?
Definition
nasal obstruction, epistaxis, midfacial destructive lesions
Term
A "lethal midline granuloma" is characteristic of...
Definition
extranodal NK/T-cell lymphoma, nasal type
Term
Where can extranodal NK/T cell lymphoma, nasal type disseminate to?
Definition
skin, soft tissue, Gi, testis (marrow involvement very uncommon)
Term
What virus is associated with extranodal NK/T-cell lymphoma, nasal type?
Definition
EBV
Term
How do you treat extranodal NK/T cell lymphoma, nasal type?
Definition
radiotherapy
Term
What is the gross growth pattern of extranodal NK/T-cell lymphoma, nasal type?
Definition
iffuse infiltrate with angiocentric and angiodestructive pattern. Causes vascular destruction with necrosis
Term
What is the cytotoxic phenotype of extranodal NK/T cell lymphoma, nasal type?
Definition
CD56+, CD2+, sCD3-, cyCD3+
Term
What is the heterogenous, wastebasket cateogry of lymphoma?
Definition
PTCL
Term
Where does PTCL occur?
Definition
mostly nodal but any site can be involved. Usually generalized with marrow involvement. Blood often involved causing a leukemic presentation
Term
What are the symptoms of PTCL?
Definition
most B symptoms and poor performance status
Term
What are the paraneoplastic features of PTCL?
Definition
eosinophilia, pruritis, hemophagocytic syndrome
Term
What is the behavior of PTCL, indolent or aggressive?
Definition
aggressive
Term
What does PTCL stand for?
Definition
peripheral T cell lymphoma, unspecified
Term
What is the pathognomic morphologic feature for PTCL, U?
Definition
there is none
Term
What CD markers are positive for PTCL, U?
Definition
CD2, CD3, CD5, CD7 (T cell markers)
may drop out CD5 or CD7
Term
What does NLPHL stand for?
Definition
nodular lymphocyte predominant HL
Term
What percent of all cases of HL are nodular lymphocyte predominat HL (NLPHL)?
Definition
5%
Term
What are the different types of classic HL and their percent of incidence?
Definition
nodular sclerosis (NSHL)- 70%
mixed cellularity (MCHL)- 20-25%
lymphocyte rich - 5%
lymphocyte depleted - <1%
Term
Which LN are affected by nodular lymphocyte predominant hodgkin lymphoma (NLPHL)?
Definition
high cervical, axillary or inguinal nodes (rare in mediastinal, splenic, BM rare)
Term
What is the age distribution of NLPHL?
Definition
30-50 years
Term
What is the M:F ratio of nodular lymphocyte predominant hodgkin lymphoma (NLPHL)?
Definition
3:1
Term
What percent of NLPHL is in stage I or II at diagnosis?
Definition
80%
Term
What is the behavior/course/prognosis of nodular lymphocyte predominance hodgkin lymphoma?
Definition
indolent, remains responsive to therapy usually, early stage prognosis excellent, advanced stages have unfavorable outcomes. Can progress to LBCL
Term
What are "popcorn cells"?
Definition
characteristic cells of nodular lymphocyte predominance hodgkin lymphoma that are CD20+, CD15-, and CD30-
Term
What age is common for classic hodgkin lymphoma?
Definition
bimodal incidence peak: 15-30 and >55 yoa
Term
What percent of CHL exhibit EBV?
Definition
50%
Term
How does HL present?
Definition
painless enlargement of nodes or mediastinal mass
Term
Which LN are affected by HL?
Definition
cervical, mediastinal, axillary, periaortic regions
Term
What percent of patients with hodgkin lymphoma have B symptoms?
Definition
40% (older patietns and young children HL have advanced disease with B symptoms)
Term
What are B symptoms?
Definition
fever > 38 degrees C, drenching night sweats, unexplained loss of >10% of body weight in the previous 6 months
Term
What is teh most important prognostic factor for HL?
Definition
disease stage
Term
What is the treatment for HL?
Definition
irradiation in localized disease, chemotherapy with disseminated
Term
Long-term survivors of HL have increased risk of..
Definition
second malignancy
Term
What is stage 1 HL?
Definition
involvement of single lymph node region (I) or single extranodal site (IE)
Term
What is stage two HL?
Definition
involvement of two or more lymph node regions on the same side of diaphragm
Term
What is stage III HL?
Definition
involvement of LN regions on both sides of the diaphragm
Term
What is stage IV HL?
Definition
multiple foci of involvement of one or more extralymphatic organs or tissues
Term
Each stage of HL can be divided into A or B, which means...
Definition
A menas absence of symptoms; B means presence of symptoms
Term
How do you diagnose HL?
Definition
presence of reed sternberg or hodgkin cells in an appropriate cellular background.
Term
What does EMA stand for?
Definition
epithelial membrane antigen
Term
What is the immunophenotype of nodular LP?
Definition
CD45+, CD20+, EMA+
CD30-, CD15-
Term
What is the immunophenotype of classic HL?
Definition
CD30+, CD15+
CD45-, CD20-, EMA-
Term
What age/gender does nodular sclerosis affect?
Definition
teenage and young adults (median age 28), M:F::1:1
Term
At what stage does nodular slcerosis HL present at?
Definition
I and II common
Term
What is a common presentation of nodular slcerosis?
Definition
dyspnea due to frequent mediastinal involvement
Term
What characterizees nodular sclerosis HL?
Definition
collagen bands in node and lacunar RS cells
Term
How does the prognosis of nodular sclerosis HL compare to mixed cellularity or lymphocyte depleted?
Definition
prognosis of nodular sclerosis HL is better than mixed cellularity or lymphocyte depleted
Term
What percent of hodgkin lymphomas are lymphocyte rich?
Definition
~5% of hodgkin lymphomas
Term
What type of HL has the highest median age?
Definition
lymphocyte rich CHL
Term
What is the gender preferance of lymphocyte rich?
Definition
70% male
Term
What HL has the best suvival rate of all clasical HL?
Definition
lymphocyte rich CHL
Term
Which nodes are involved in lymphocyte rich CHL?
Definition
peripheral nodes commonly involved
Term
What is histological description of mixed cellularity CHL?
Definition
mix of lymphocytes, plasma cell, eosinophils, and RS cells
Term
In what patient population is mixed cellularity CHL most common?
Definition
pts with HIV and in developing countries
Term
What is the age/gender preference of mixed cellularity CHL?
Definition
37 yoa; 70% male
Term
Which HL has uncommon mediastinal involvement?
Definition
mixed cellularity CHL
Term
What stage does mixed cellularity CHL present as?
Definition
stage III or IV
Term
Does mixed cellularity CHL commonly have symptoms?
Definition
yes (b symptoms)
Term
What is lymphocyte depleted CHL?
Definition
diffuse form of CHL rich in RS cells and depleted of non-neoplastic cells; most often diagnosed in HIV+ patietns whose nodes are depleted of lymphocytes
Term
What is the median age and gender preference of lymphocyte depleted CHL?
Definition
age= 37 years, 75% male
Term
What is the presenting stage and symptoms of Lymphocyte depleted CHL?
Definition
usually advanced stage (70%) and B symptoms (80%)
Term
T/F Course of lymphocyte depleted CHL is comparable with other CHL of similar stage
Definition
true
Term
What are the functions of the spleen?
Definition
phagocytosis of blodo cells and particular matter (filtration of unwanted elements from the blood); antibody production; hematopoiesis (lymphoreticular cells and sometimes hematopoietic cells (extramedullary hematopoiesis in some disease staes), sequestration of blood elements (reserve pool and storage site)
Term
What percent of total platelet mass can be squestered by the spleen?
Definition
90%
Term
Splenomegally may cause a condition called ___ characterized by...
Definition
hypersplenism; anemia, leukopenia, thrombocytopenia
Term
Splenic infarcts are most often caused by...
Definition
emboli, most often from heart valves (may be septic)
Term
Regardless of the cause of a splenic infarct, most splenic infarcts occur in..
Definition
enlarged spleens b/c blood supply is tenuous and easily compromised
Term
What causes splenic insufficiency?
Definition
splenectomy, autoinfarction (sickle cell dz)
Term
Splenic insufficiency leads to increased susceptibility to sepsis by __________.
Definition
encapsulated bacteria (pneumococcus, meningococcus, H. influenzae)
Term
What are accessory spleens?
Definition
small and spherical extra spleens located anywhere in the abodminal cavity
Term
What is the clinical significance of accessory spleens?
Definition
if overlooked then the benefit of therapeutic splenectomy may be reduced or lost (hereditary spherocytosis, immune thrombocytopenia)
Term
What cells are present in the thymus?
Definition
naive T cells, thymic epithelial cells, b cells (may form thymic b cell lymphoma), dendritic cells
Term
What are thymic cysts?
Definition
uncommon rarely exceed 4 cm, usually discovered at autopsy or during surgery
Term
What developemental disorder is associated with aplasia of the thymus?
Definition
diGeorge syndrome
Term
What are the characteristics of DiGeorge syndrome?
Definition
severe defects in cell-mediated immunity, part of 22q11 deletion syndrome, recurrent viral and fungal disease, parathyroid hypoplasia, congenital heart disease
Term
What causes thymic (follicular) hyperplasia?
Definition
chronic inflammatory and immunologic states; most frequently seen with myasthenia gravis but also seen in graves' disease, lupus, RA, and other autoimmune disorders
also seen in neoplasms
Term
Which neoplasms are associated with the thymus?
Definition
germ cell tumors, lymphomas, carcinoids, and thymomas
Term
What age/gender is typical of thymomas?
Definition
adults >40 yoa M=F
Term
Where do thymomas occur?
Definition
anterior superior mediastinum (like lymphomas)
Term
What are the subtypes of thymoma?
Definition
cytologically benign and non-invasive; cytologically benign and invasive thru the capsule (may metastasize), cytologically malignant (thymic carcinoma) which usally invades adjacent structures and are difficult to remove
Term
What is the clinical presentation of thymomas?
Definition
impingement on mediastinal structures; myasthenia gravis and other autoimmune dzs such as pure red cell aplasia and graves'
Term
Why are AI dzs associated with thymomas?
Definition
long-lived CD4+ and CD8+ are likely to be associated with AI dzs
Term
How do you treat thymoma associated AI dzs?
Definition
removal of the thymoma can resolve the AI d/o
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