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White Blood Cells, Bone Marrow, Lymphoid Tissues

A blast cell or stem cell is characterized (generically) with what immunophenotype? CD34 or Tdt (or CD10 in ALL)
a maturing cell is characterized (generically) with what immunophenotype, relevant to each cell type: B cell, granulocyte, T cell? B cell (sIg), granulocyte (CD15), T cell (CD4/CD8)
A committed T cell will express what surface markers? Either CD4/CD8, CD7, CD2, and will rearrange its TCR genes.
If a virgin lymphocyte moves to the peripheral blood and colonizes the mantle zone where it encounters antigen, it will then form a germinal center, now termed a ______ cell. small non-cleaved cell
what are small cleaved lymphocytes? a lymphocyte that has been antigenically stimulated (small non-cleaved) and then has successfully undergone somatic mutations that allow it to survive and bind the antigen with higher affinity, thus avoiding destruction by macrophages
CD10 and CD34 indicate what? an immature lymphoblast
If flow cytometry detects IgG, IgA, or IgE on a the surface of lymphocyte, what does this mean? It is either at or beyond the germinal center
The CDR (complementary determining regions) of an Ig molecule are made up of what? variable regions that bind antigen encoded by the VDJC complex
In flow cytometery, the "side-scattered light axis" determis what? nuclear complexity
In flow cytometry, "forward-scattered light axis" determines what? cell size
What immunophenotype would you expect to find for a neutrophil or monocyte? You wouldn't run flow on these, they are morphologically distinct enough
What are the common T cell markers? CD1-->CD10 basically
What are the common B cell markers? CD19, CD20, 21, 22 (20's)
what are the common myeloid markers? CD13, CD33, CD34, CD11, CD15, CD16, CD18
What generically characterizes a neoplastic process in a lymphnode, for example.? Destruction of architecture, abnormal/atypical/pleiomorphic cells that are CLONAL.
You suspect a neoplastic process in a lymphnode based on clinical symptoms. The architecture isn't greatly disrupted and the cells don't look grossly abnormal. How can you rule out? You would look for CLONALITY. In B cells you would look for monotypic kappa or lambda restriction. In T cells, it's harder, you would have to do genetic analysis of the antigen receptor molecules (TCR's).
Where are resting lymphocytes found in a lymphnode? mantle zone
What is a "Tingible-body" a macrophage that has digested an unsuccesful lymphocyte
what is a good marker to determine the mantle zone lymphocyte from the germinal center lymphocyte? Ki-67 (a proliferation marker, highlighting germinal center cells)
A lymphocyte comes back Ki-67 positive? where did it come from? germinal center
In general terms, Bcl-2 is only expressed by what? also known as what? resting lymphocytes. anti-apoptosis protein
what is the basic cellular difference between acute and chronic leukemias? ACUTE (immature cells) CHRONIC (mature cells)
What is the major lymphoma of granulocytes? THERE IS NO LYMPHOMA FOR GRANULOCYTES, lymphomas involve lymphoid cells
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of B LYMPHOBLASTS? ALL
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of VIRGIN B LYMPHOCYTES? CLL
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of MANTLE ZONE LYMPHOCYTES? mantle cell lymphoma
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of FOLLICULAR CENTER LYMPHOCYTES? follicular lymphoma
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of PLASMA CELLS? plasma cell myeloma (MULITIPLE MYELOMA)
What is the most likely neoplastic process in a smear with stage of cell maturation that is composed of MARGINAL ZONE LYMPHOCYTES? marginal zone lymphoma (includes MALT)
In the BM, what cell architecture normally leans toward a neoplastic process? Think about normal histology? Normally BM has 50-50 cell:fat ratio. Normally cells are in equal proportions of (megakaryocytes, immature RBCs, immature myeloid cells). So if you see replacement of any of the normal=neoplasm
what is the morphology of a blast? blasts have NO-Home (NOH): nucleolus, open chromatin, high N/C ratio
What are the main findings if hematopoietic tissues have been replaced with neoplastic processes? anemia (fatigue), thrombocytopenia (bleeding), leukopenia (fever, infection)
The majority of acute leukemias involve which cell? pre-B cells
Acute Leukemia invovlves what tissues, regarding different cells involved? pre-B cells (extensive BM/LN); pre-T cells (LN or mediastinum)
ACUTE LEUKEMIA is most often seen at what age for each cell type? preB (peak age=4); preT (adolescent males)
Of the 2 types of ALL, which cell type produces a greater LAD and splenomegaly? pre-T ALL
A 15 yo male ptc with anemia, neutropenia, and thrombocytopenia. His complains of "pains in his bones". PE reveals splenomegaly. You notice mediastinal masses and airway/vessel obstruction due to an enlarged thymus. What is your guess on the immuno? CD1-->CD8, TdT+
What is the immunophenotype for preB cell ALL? TdT+, CD10+, CD19+, no sIg, CD22+
ALL has a tendency to metastasize where, somewhat uniquely? CNS and testicles
What is the treatment for ALL? prognosis? Tx: aggressive chemo + CNS prophylaxis
You diagnose 4 children with ALL. Annie is 1. Bob is 3. Chris is 15 (and has the Ph chromosome), David is 15 (no Ph chr). What are their prx? Annie has the worst prognosis (<2), then Chris (>10, Ph chr bad), then David (>10, no Ph), then Bob (>2, <10)
What determines a favorable prx for ALL? 2-10yo, hyperdiploidy, t(12;21)
what determines an unfavorable prx for ALL? <2, >10, adult, Ph chr [t(9;22).
How commonly does the Philadelphia (9;22) translocation occur in ALL causing an unfavorable prx? 20% of ALL pts
What normally characterizes a CLL/SLL neoplastic process? proliferation centers (prolymphocyte aggreagates; pale areas), lymphnode effacement, absolute lymphocytosis (>4000), BM infiltration (focal/extensive)
CLL/SLL immunophenotype? CD19, CD20, CD5 (T cell coexpression), sIg (mature lymphocytes), pregerminal center (sIgM)
what is the typically ptc for CLL/SLL? 60yo, usually asymptomatic, LAD & fatigued (50%), with lymphocytosis (>4000-->200,000) with bacterial infx, and autoimmune hemolysis
autoimmune hemolysis often associated with? CLL/SLL--> smudge cells
smudge cells CLL/SLL due to autoimmune hemolysis
what is the overall suvival for CLL/SLL? 4-6 years
You get a dx back from HemOnc on your patient: early stage CLL/SLL. you know you don't treat CLL/SLL until later stage. what do you need to know before you decide NOT to treat? ZAP-70 status. ZAP70+ equals CLL/SLL that's more aggressive and needs treated earlier
What is ZAP-70 (Zeta Chain Activated Protein 70kDa)? a protein that can be overexpressed in CLL/SLL which indicates a more aggressive form of the disease, that unlike less aggressive CLL/SLL, needs treated early; it's a surrogate marker for UNMUTATED STATUS of the VARIABLE regins of the Ig HEAVY CHAINS
Molecular analysis shows that the variable regions of your patients HEAVY chains are mutated in their variable regions. This means they are ZAP70 (+/-?) MUTATIONS=Zap70 negative, less aggressive form of the disease
What types of cells may be seen in histologic section of someone with CLL/SLL? smudge cells (ruptured spherocytes), spherocytes--> both due to autoimm hemolysis, NRBCs
proliferation centers CLL/SLL
what is the immunophenotype for CLL/SLL? CD19, CD20, CD23, CD5, sIg
what translocations are associated with CLL/SLL? THERE ARE NONE; rather deletions and trisomy
The general prx for CLL/SLL is ______? What can make the prx worse? CLL/SLL can undergo prolymphocytic transformation (15-30% of the time) or DLBCL (Richter Syndrome, 10% of the time) which shortens the prx to 1 year.
ALL cases of CLL invovle____? SLL most likely invovles_____? Since they are so similar, what other differences are there? bone marrow; intestinal infiltrates; other differences (CLL=BM+leukocytosis) (SLL=BM+leukpenia)
Histologically, you get a Lymphoma dx that resembles either CLL/SLL or mantle cell lymphoma. How do you tell the difference? both will have CD5, but only CLL/SLL will have CD23. Mantle cell lymphoma is CD23 neg. You don't put M. Jordan on the mantle!
Immunophenotype for Mantle cell lymphoma? CD19+, CD20+, CD5+, CD23-, sIg, sIgM or IgD (pregerminal)
Which is clinically more aggressive? CLL/SLL or mantle cell lymphoma? mantle cell lymphoma (M.Jordan gives you a little more strength)
Mantle cell lymphoma is associated with what gene problems? t(11;14) with bcl-1 transolocation and cyclin D1 overexpression
what is the neoplastic process with cyclin D1? it overcomes the G1-S block by phosphorylating retinoblastoma increasing progression thru cell cycle
overexpression of cyclin D1 causes what lymphoma? what transolcation causes this> t(11;14); Mantle cell lymphoma
what is the typical ptc for mantle cell lymphoma? MALE, 50-60, generalized PAINLESS LAD, lymphomatoid polyposis ocassionally
lymphomatoid polyposis ocassionally associated with? mantle cell lymphoma
what is the most likely cell etiology for mantle cell lymphoma and why? naive B cell, because of NO somatic mutations
what % of mantle cell lymphomas involve the PB? 20-40%
what is the most likely cell etiology for CLL/SLL and why? is there a reason for distinguishing? both naive B cell (no somatic mutations) and memory B cell (somatic mutations). yes, NAIVE B CELL CLL/SLL has a worse prx
what is Richter Syndrome? how frequent? it occurs in CLL/SLL in 10% of pts. it's when the CLL/SLL cells transfrom to a diffuse large B cell lymphoma, with a worse prx (1 year)
what is the frequency of follicular lymphoma in terms of NHL? 50%
what is the immunophenotype of follicular lymphoma? CD19+, CD20+, CD5-, CD10+, sIg+, Bcl2+, Bcl6+,
what is the treatment for follicular lymphoma? no treatment
Is there anything that can make a follicular lymphoma prx worse? transformation to DLBCL (30-50%)
what is the typical follicular lymphoma prx? indolent course 7-9years
what is the gene association with follicular lymphoma? t(14;18) which places Bcl2 next to IgH which prevents apoptosis
what distinguishes a follicular lymphoma GC cell from a non? normal GC cells don't express Bcl2
what is the typically ptc for follicular lymphoma? painless LAD, lymphocytosis <20,000 (10%), M=F, middle age
what cells are seen histologically in follicular lymphoma? small cleaved cells *majority (centrocytes), centroblasts (large non-cleaved), B-blasts (small non-cleaved)
What is associated with an IgH-Bcl2 transloaction? t(14;18) Follicular lymphoma
what is the cell etiology in follicular lymphoma? pre-germinal center (no somatic mutations in teh V region of the Ig heavy chain genes/IgM); post germinal (isotype switching IgGAE/somatic muations+)
DLBCL comprise ____% of NHL in US? 20%
what is the immunophenotype of DLBCL? CD19+, CD20+, sIg+, maybe CD10+, Bcl6+
are there any gene associations with DLBCL? 30% have t(14;18) being derived from follicular lymphoma
what can progress to DLBCL? CLL/SLL or Follicular cell lymphoma
Is there anything that gives DLBCL a favorable/unfav prx? FAVORABLE (3q27 translocation) making them germinal center like) UNFAV (antiapoptotic signals: bcl2, FLIP, MUM-1)
bcl2, FLIP, and MUM1 may be genetically profiled to which type of NHL? DLBCL--> unfav prx
what is the typically ptc of DLBCL? 60yo M, clinically symptomatic, with a rapidly enlarging symptomatic mass of a single node
what is the prx for DLBCL? 60-80% remission, 50% cure with chemo; aggressive if untreated
predisposing factors for DLBCL? CLL/SLL (Richter Sydrome), Follicular Lymphoma [t(14;18); immunodeficiency (HIV: EBV/KSHV)
What are the types of Burkitt's Lymphoma? Endemic (African--latent EBV infx), Non-endemic (sporadic), and HIV associated
Histology of Burkitt's Lymphoma? starry night sky, increased mitotic index
what is the translocation associated with BL? t(8;14) myc/IgH
what is the association with EBV and BL? all pts with African BL have EBV infx, where 25% of non-endemic have
Most Burkitt's lymphomas are extranodal, hardly leukemic or in BM. Where are the most common extranodal sites. Take into account different types/ African (mandible); Non-endemic: ileocecum
who is typically affected by BL? young adults and children
What are frequent NHL in children? lymphoblastic lymphoma (ALL) and Burkitt's Lymphoma
What is the M component? a monoclonal immunoglobulin secreted by a neoplastic plasma cell, located in the peripheral blood
What is Bence-Jones Protein? free light chains in the urine from a monoclonal gammopathy from a plasma cell neoplasm
Monoclonal gammopathy resulting from a plasma cell dyscrasia causes what problems? multiple myeloma, Waldenstrom Macroglobulinemia, Heavy chain ds, primary amyloidosis, MGUS
what is another name for multiple myeloma in the skin? plasmacytoma
what is the most common symtpom of monoclonal gammopathy? multiple myeloma
what are the clinical characteristics of multiple myeloma? multiple punch-out lesions (osteolytic) in skeletal system, which can spread to LN or skin, M>F,AA, older adults, increase serum IL6, usually multifocal in skin, pathologic frx, osteoclastic (hyperCa2+:neurologic sympx, confus, constipation), peak 60yo
where are the typical osteolytic lesions in multiple myeloma? vertebrae, skull, pelvis
what is the typical ptc for multiple myeloma? 60yo AAM with pathologic frx
Histologic feature of multiple myeloma? increased plasma cells in marrow, often move medullary to cortex, abundant "PLASMABLASTS")
what is the mean age for multiple myeloma? 60yo
A 65yo AAM ptc with backpain. He complains of abd pain and constipation. He feels sluggish, and "not right in the head." Labs reveal normocytic, normochromic anemia and renal insufficiency. Elevated neutrophils. What other tests? Dx? evaluate elevated serum IL6, hypercalcemia, BJP (to see if above 6mg), serum Ig (to see if above 3mg); xray (osteolytic lesions); MULTIPLE MYELOMA
What is Waldenstrom Macroglobulinemia? A result of monoclonal gammopathy, due to increased IgM that causes hyperviscosity, most commonly seen in lymphoplasmacytic lymphoma; may cause visual impairment, bleeding (combines w/clotting factors), Raynauds
A pt comes in complaining of lower extremety and digit pain, visual problems. Upon futher questioning, he's bleeding more easily. Labs reveal increased IgM. Dx? Waldenstrom Macroglobulinemia--> possibly due to multiple myeloma or lymphoplasmacytic lymphoma
What would you expect to see on serum electrophoresis to indicate monoclonal gammopathy? >3mg of Ig; below this it could be MGUS where 10% progress to MG
What % of MGUS progress to monoclonal gammopathy? what characterizes MGUS? 10%, <3mg of monoclonal Ig, M component in blood but no sympx, most are benign. NO BJP
You have a pt that ptc with no propbelms but you incidentally find an elevated M component (monoclonal gammopathy of 2.4mg. Is this significant? YES. This would be dx as MGUS. It would impt to monitor his levels bc 10% of MGUS pts progress to MG.
Why does monoclonal gammopathy cause a primary amyloidosis? bc plasma cells secrete IL6, which stimulate liver protein production of protein which is converted in the blood to amyloid
what are the majority of immunoglobulins in multiple myeloma? IgG (55%) and IgA (25%)
What defines a slow progressing, indolent multiple myeloma present in 3-5%? solitary lesions
What is the type of B cell MALT lymphoma (MALTOMA)? Marginal Zone Lymphoma
Where does a maltoma (B cell type) generally locate? (Marginal Zone Lymphoma) affects mucosa (salivary glands, stomach)
In the stomach, a B cell MALTOMA or Marginal Zone Lymphoma is associated with what? Helicobacter pylori
MZL (B cell MALTOMA) often arises bc of what? in the setting of chronic inflammation. starts as reactive-->mut-->monoclonal B cell; often associated with autoimmune ds's (Sjogren's/Hashimoto's Thyroiditis)
What is the typical progression of MZL (B cell Maltoma)? they start as a reactive process to inflammation (may be autoimmune, infx), remain localized for long periosd, become system, gain mutations, and progress to monoclonal B cells, and may even go to DLBCL
Sjogren's disease/Hashimoto's Thryroiditis MZL (B cell MALTOMA)
what is the predicatable order of spread in Hodgkins (B cell) Lymphoma? node, spleen, liver, marrow, extranodal
HL is characterized morphologically by the presence of _______ cells, which cause what? REED-STERNBERG CELLS which cause an accumulation of reactive lymphocytes, histiocytes, and granulocytes
What are the 5 types of HL? nodular sclerosis, mixed cellularity, lymphocyte rich, lymphocyte depletion (all the classic types) and lymphocyte predominance
Why are only 4 types of HL considered classic? This is bc the RSC's demonstrate the same immunophenotype so they're lumped together; Lymphocyte predominant type has RSC's with a B cell IP, which is not the case with classic HL.
Why is staging of HL important? Bc the disease predictably spreads, knowledge of stage allows prediction of prx and for therapy.
Localized HL is trx with what? radiotherapy
disseminated HL is trx with what> chemotherapy: MOPP (mechloraethamin, vinicristine, prednisone, procarbazine)
Nodular sclerosis type of HL females, mediastinal masses, most common HL, lacunar cells, collagen bands, lower cerv/supraclav/mediastinal LN, rarely ass with EBV, young adults, CD15/30+, excellent prx
young females with mediastinal masses, concerning HL nodular sclerosis
Mixed Cellularity? males, older age, systemic symtpoms, advanced tumor stage, T cell pred background, strong EBV-ass in RSCs(70%), CD15/30+, mononuclear variant+RSCs, diffuse effacement of LN, 25% of cases, good prx
In HL, _____ comprises 75% of cases, whereas ____ comprises 25%. nodular sclerosis, mixed cellularity
Which HL involve youngsters? nodular sclerosis, lymphocyte predominant
Lymphocyte Rich type of HL? uncommon form of classic HL, reactive lymphocytes make up the majority of cell infiltrate, EBV ass (40%), good prx
Lymphocyte Depletion Type of HL? least common form <5%, paucisity of lymphocytes and a relative abundance of RSCs or their variants, older pts, HIV+, EBV ass, advanced stages, systemic symptoms, outcome less favorable
Lymphocyte Predominant Type of HL? uncommon (5%), male, usually younger than 35yo, popcorn cell (RSC--LH variants), B cell like, CD20+, CD15/30-, background of Blymphocytes only, RADIOSENSITIVE, localized ds, germinal center B cell origin (Bcl 6, somat mut), NO EBV, LAD, recur, excel prx
EBV is frequently involved in the episome of the RSC in HL, esp in ______? mixed cellularity
What do RSCs secrete for specific cell backgrounds? cytokines: IL5 (eosinophils), TGF-beta (fibrosis in NS)
What does RSC secrete that causes fibrosis in nodular sclerosis? TGF-beta
what attracts eosinophils, released by RSCs in HL? IL5
EBV episome is associated with transformation in HL in which types? it is not associated with which type? nodular sclerosis and mixed cellularity; lymphocyte predominant
Which types of HL are more likely to present with disseminated disease (liekly to have advanced stage and night sweats/weight loss? mixed cellularity, lymphocyte depletion
What is a RARE paraneoplastic syndrome specific to HL? pain in LN upon consumption of alcohol
Pt has pain in LN when drinking alcohol? HL (paraneoplastic syndrome)
How are HL and NHL different? HL (localized, contiguous spread, neck/mediastinum, rarely extranodal, MOPP tx) NHL (disseminated, non-contiguous spread, any site involved, usually extranodal, CHOP tx)
what helps T cells mature in the thymus? Hassals corpuscles
How are T cell neoplasms different than B cell neoplasms? almost always have genralized adenopathy, more aggressive, have higher rate of relapse than B cell NHL
T cell neoplasms comprise what % of NHL? 20%, higher in ASIA
Cutaneous T cell Lymphoma (CTCL)? lymphocytes highly irregular, cerebriform; SKIN (mycosis fungoides); blood (Sezary syndrome: exfoliative erythroderma); CD4, prefers skin; surv 8-9yrs
Adult T cell Leukemia/Lymphoma ATCL? HTLV1 virus, CD4+, skin lesions, hypercalcemia, clover leaf cells, rapidly fatal (1year) in most despite tx, may also demyelinate brain
ALCL (anaplastic large cell lymphoma)? express CD30, bear t(2;5), Hallmark cell (horseshoe nuclei), ALK rearrangements, worse prx without ALK (adults)
What are the three categories of myeloid neoplasms? AML; myelodysplastic syndromes; chronic myeloproliferative disorders
Most of the myeloid neoplasms transfrom to more aggressive froms of disease (AML). ________ may transform to ALL. CML, a type of chronic myeloproliferative disorder
AML are classified based on degree of maturation. Explain the subclasses. MO-M3 (Myleocytic); M4-M5 (Monocytic); M6 (Erythrocytic); M7 (Megakaryocytic)
M0-M3 AML involves what cells? myelocytic/granulocytic
M4-M5 AML involves what cells? monocytic
M6 involves what cells in AML? erythrocytes
M7 AML inolves what cells? megakaryocytes
chronic myeloproliferative disorders include what subcategories? CML, polycythemia vera, essentail thrombocytopenia, myelofibrosis (agnogenic myeloid metaplasia)
Myelodysplastic syndromes include subtypes? refractory anemia, sideroblastic anemia, chronic myelomonocytic leukemia
M3 AML subtype? Acute Promyelocytic leukemia
What is used to dx AML? >20% blasts in BM, granules in blasts with auer rods, myeloperoxidase +, flow cytometry markers (CD33, CD34)
what are the clinical features of AML? marked leukocytosis (usually >100,000)
AML-M3 Acute Promyelocytic Leukemia typically ptc? bleeding due to thrombocytopenia
What is found on histology in AML-M2 Acute Promyelocutic Leuk? numerous blasts as well as abn dumbell-shaped promyelocytes with prominent azurophilic granules; blasts may contain Auer rods (Faggot cells)
What are Faggot Cells are where are they seen? Faggot Cells are blasts with numerous auer rods in APL (AML-M3)
AML-M3 (APL) results from what? t(15;17) involving retinoic acid receptor and PML genes; RAR allows myeloid differentiation, but becomes defective when transloc to PML, thus preventing differentiation.
You have a patient with AML-M3 (Acute Promyelocytic Leukemia) dx after viewing blasts with dumbless shaped promyelocytes and faggot cells on slide. You know this is from a t(15;17). Is this trx? YES. You can treat with ATRA (all trans retinoic acid) which overcomes the block in differentiation, converting accumulated promyelocytes to bands/neutrophils. May need to combine with other drugs, normally not effective alone.
You have a pt with AML-M3 Acute Promyelocytic Leukemia and you treat with ATRA (all trans retinoic acid). What will you see rise in his peripheral blood? bands/neutrophils, but they are short lived
ATRA (all trans retinoid acid) used to treat AML-M3 Acute Promyelocytic Leukemia
t(15;17) AML-M3 Acute Promyelocytic Leukemia
AML-M5 Acute Monoblastic Leukemia usually ptc? features of any acute leukemia, but also with skin and gingival infiltrates called (Leuk. cutis)
In AML, what is the difference between a MYLEOBLAST and a MONOBLAST myeloblasts (have 2-4 nuclei, and Auer Rods); MONOBLASTS (lack auer rods, peroxidase neg)
Describe the relationship between the number of leukemic cells in the PB in AML? IT's VARIABLE. Most blast counts are below 10,000 (which is normal) in 50% of the cases (which would appear as a Leukemoid reaction). They may be above 100,000. Or you have NO PB involvement at all (aleukemic leukemia)
Which AML subtype usually involves the skin and gingiva? AML-M5 Acute Monoblastic Leukemia
Many gene translocations have been associated with AML, giving variability to prx. Which rearrangements are favorable? intermediate? unfavorable? FAVORABLE [t(8;21), inv16] INTERMEDIATE [t(15;17)] UNFAVORABLE [t(11q23]
AML and ALL usually present with the same symptoms. What is the main difference? AML usually has a much more predominant thrombocytopenia and thus BLEEDING
What characterizes AML-NOS (not otherwise specified)? Subgroup MO-M7 with no recurrent chromosomal rearrangement
In Myelodysplastic syndrome, what are the cytogenetic abnormalities? monsomy 5, monosomy 7, trisomy 8
what is the risk factor with myelodysplastic syndrome? it has increased chance to turn into AML
Histologically, what do you see in Myelodsyplastic syndrome? pancyto abnormalities (RBC=ring sideroblasts) (granulocytes=PseudoPelgerHuet cells) (abn megakaryocutic maturation)
Ring sideroblasts? where do you find? erythroblasts with iron laden mitochondria visible as perinuclear granules in Prussian blue stained aspirates or biopsies; myelodysplastic syndrome
Pseudo-Pelger Huet Cells? where do you find? neutrophils with 2 nuclei; myelodysplastic syndrome
myelodysplastic syndrome--ptc? >60yo, pancytopenia, weakness, infx, hemorrhages
how are myelodysplastic syndromes classified? by the percentage of blasts--> refractory anemia (<5%), sideroblastic anemia (<5%); refractory anemia with blast excess (5-20%)
Chronic Myelomonocytic Leukemia (AML-M4) may sometimes be confused with Myelodysplastic syndroome. How do you tell the difference? In MDS, it will be t(9;22) negative
You have a pt with MYELODYSPLASTIC SYNDROME, what determines his prx? degree of cytopenia and whether or not he will develop AML (which is proportional to BLAST COUNT) HIGHER BLAST %=HIGHER RISK OF AML=worse prx
what determines the risk of AML transformation in a pt with myelodysplastic syndrome? % of blasts (increase %, increase risk, worse prx)
How do you treat a 89yo man with myelodysplastic syndrome? NO CHEMO. Older pts are treated supportively with abx and blood product transfusions
When you have a chronic myeloproliferative disorder that is pan myeloid, what is the disease? Agnogenic Myeloid Metaplasia (MYELOFIBROSIS)
In CHRONIC MYELOPROLIFERATIVE DISORDERS the defective cell typically involes pleuripotient stem cells that increase all myeloid lineages. What is the exception? CML (stem cells only give rise to lymphoid and myeloid)
How are AML and CML different if both are involving precursors? CML allows terminal differentiation, where AML doesn't.
All forms of chronic myeloproliferative disorders progress to AML, but ___________ does so invariably? CML
What is the typical age of ptc for CML? adults (40-50)
t(9;22) CML: juxtaposition of ABL on chr9 to the BCR gene on chr22 increasing cell div, inhibiting apoptosis at the pluripotent stem cell level
How do you treat CML? ABL-BCR kinase inhibitors--> STI 571 (IMATINIB MESYLATE)
STI 571 (Imatibib Mesylate)? What is it used in? It block the ATP binding domain which doesn't allow autophosphorylation of tyrosine kinase residues disrupting STPs, which may cause remission in CML (ABL-BCR kinase inhibitor)
You have a pt with a leukocytosis which you may attribute to CML (assuming you have no ABL-BCR studies). What would lead you to believe that its a reactive leukocytosis? infl, infx, stress, increased neutrophils/bands, less metamyelocytes, much less myelocytes or promyelocytes
What is the normal range for leukocytes? 4500-10,000 x10^9
Leukocytosis is defined as? >10,000 WBC
Leukopenia is defined as? <4500 WBC
What are the charact of Superior Vena Caval Syndrome? thymoma, medistinal fibrosis (slcerosisng mediastinitis), lymphoma, small cell CAR of lung
You have a pt with neck adenopathy. What should be considered? MONO, Toxo (esp if young), oral CA metastasis, lymphoma (HL/NHL)
what is the normal platelet count? 150,000 to 400,000
You have a pt with epistaxis. Consider the causes. DECREASED PLATELETS: decreased production (lymphoma), increased destruction (splenic dstrx, DIC, autoimmune)
hypergranular promyelocytes? M3-AML (APL) t(15;17)
What are the causes of splenomegaly? mono, CMPD (myelofibrosis, CML), lymphoma, cirrhotic congestion
In Chronic Myeloproliferative Disorder, which cause splenomegaly? CML, myelofibrosis (OLDER PEOPLE)
You have 69yo male who ptc with splenomegaly. He is EBV neg. LFT normal. No hepatomegaly. No cardiac problems. Differential? Because he's older, you would consider CMPD (myelofibrosis, CML)
essential thrombocythemia (in CMPD) is characterized by what platelet count? >600,000
You have a patient with >600,000, what is the dx? Must investigate bc all CMPD's can increase platelets, ESSENTIAL THROMBOCYTOTHEMIA IS A DIAGNOSIS OF EXCLUSION
What is one characteristic symptom of Essential Thrombocythemia? ERYTHROMELAGLIA (burning of hands and feet due to thrombocyte congestion)
sea blue histiocytes CML
dacryocytes (tear drop shaped erythrocytes) primary myelofibrosis (agnogenic myeloid metaplasia)--myelofibrosis damages RBC mem on its way out of BM
An erythrocyte expresses ______ when it's committed. glycophorin
When granulocytes are committed they express _______. myeloperoxidase
Explain the process of B cell maturation. BM : early preB(TdT+, CD34+, CD19+, CD10+) preB (cIg, CD20). PB: loses CD10, CD34 and TdT. Gains sIg, light chain rearrangments. Plasma cell (cIg only)
Explain CD19. B cell marker expressed from early preB up to parafollicular B. Lost in plasma cell
Explain CD20. B cell marker: expressed with sIg so in mature virgin B cell to parafollicular B cell. Not in plasma cell
pan B cell markers CD19/20
restrictive B cell marker CD10
Explain CD10. CALLA B cell marker; expressed early, lost when enters peripheral blood, rexpressed in follicular center, and lost when leaves GC.
Explain cIg. Expressed in early maturation, lost until plasma cell.
When does a B cell get HEAVY CHAINS (IgH). It's born with Heavy Chains (IgM) which isotype switch after Ag exposure in GC.
You get a histogram back based on physical properties (forward and side scattered light). Describe where the cells will fall on the histiogram. LYMPHOCYTES (least complex, smallest size) GRAN (most complex, mid size) MONO (mid complex, largest size)
You have 2 lymphocytes, >___% difference in their variable Heavy chains means that one has been exposed to antigen. >2%
CD19+ CD20- CD10+ cIg+ sIg- IgM+ no SM early B cell
CD19+, CD20+, CD10-, cIg-, sIg+, IgM+, no SM mature lymphocyte in mantle
CD19+, CD20+, CD10+, cIg-, sIg+, IgG/A+ SM= mature lymphocyte in follicular center
CD19-, CD20-, CD10-, cIg+, sIg- IgG/A+, SM+ plasma cell
Ki67 proliferation marker (germinal center) NOT in mantle bc they are not proliferating
What are the main two differences in a LN regarding lymphoma and reactive processes? REACTIVE (polarity, macrophages), lacking in lymphoma
what is the pan T cell marker? "235" CD2, CD3, CD5
you see a LN with predominance of small non-cleaved? Burkitt Lymphoma (NOT sure about this)
most common leukemia of adults CLL/SLL
pts with mantle cell lymphoma typically liver how long? 3-4 years
mantle cell lymphoma and SLL/CLL both express CD5. Which is more aggressive? mantle cell lymphoma
bcl + cyclin D translocation t(11;14)
"Wait until it's aggressive" Don't treat CLL/SLL (Rai & Binet) until aggressive, unless Zap70+ (unmutated)
what's the surface Ig in mantle cell lymphoma? IgM or D
which NHL are extremely aggressive? ALL and Burkitts Lymphoma
What is the relationship between follicular lymphoma and children? nothing....you hardly ever see FL in children (CHILDREN NEVER GO TO FLORIDA)
mature B cell with CD10 in a lymphoma? follicular lymphoma
At a high magnification, a follicular cell lymphoma will show what? cleaved cells
what's the second most common NHL? DLBCL
DLBCL has a strong relationship with what? follicular lymphoma
where do DLBCL typically show up? rapidly enlarging singl emass in WALDEYER RING (mouth) or in liver/spleen
what is the histologic picture of a DLBCL cell? vesicular chromatin, large, no granules, looks empty
Bcl6+, CD10+, CD38+ DLBCL (better prx)
how is Burkitts Lymphoma treated? used to be treated successfully with cyclophosphamide, but now requires multidrug
How large is Chris's PENIS? yeah right...
CDA chemo? tx for Hairy Cell Leukemia
according to the notes, what are the main clinical features of Lymphocyte Predominant HL? young age, radiosensitive, localized
What is the special immunophenotypic marker for anaplastic T cell lymphoma? special histological feature? CD30+, Hallmark cell
Pautrier microabscesses? where are they located in the skin? cutaneous T cell lymphoma; intraepidermal
You notice a cerebriform lymphocyte in the blood? Sezary syndrome
What is the leukemic form of Mycosis fungoides (cutaneous T cell lymphoma)? Sezary syndrome
You notice a large follicular hyperplasia in a LN. All you are told is that it's not cancer. What could be causing this? Rheumatoid arthritis, SLE, syphilis, toxo, HIV, non-specific
Langhan's cells (NOT LANGERHANS)? Tb
You notice a cell that looks like it has a "wreath-like nuclei"? Langhans cell--Tuberculosis
what is the single most important cuase of death due to infectious disease Tb
You have a pt with characteristic SARCOIDOSIS. What did this person likely have before this? What would you expect to see on Xray? What tissue site is involved 90% of the time? What other sites are freq involved? Erythema nodosum (precedes) Bilateral hilar lymphadenopathy (xray), lung (90%), LN, liver, skin, eyes (other sites)
Sarcoidosis produces _______ inflammation? granulomatous
asteroid body? sarcoidosis
suppurative granuloma of a LN (center with Neutrophils and necrosis) Hensale (cat scratch fever)
You notice a cell in the shape of a dumbell, from the myeloid line? AML-3 (Acute Promyelocytic Leukemia)
In CML, what do the cells lack? leukocyte alkaline phosphatase
Leukoerythroblastic rxn in myeloid cells? MYLEOFIBROSIS
what are the main things for myelodysplastic syndrome? no peripheral blood, ineffective hematopoiesis, increased risk for AML, ring sideroblasts, chr abn (-5,-7, +8)
Myelodysplastic syndrome causes abnormal cell morphologies. "dysplastic maturation." explain this. RBC (unusual iron accumulation: ring sideroblasts) granulocytes (neutrophils without proper segmentation: pseudo pelger-huett cells; granulocytes without granules)
Birbeck granules? langerhan cell histiocytosis
Letter-Siwe Syndrome? Langerhan cell histiocytosis: Acute, children, skin (trunk/scalp), rapidly fatal if untrx, multisystem multifocal
Eosinophilic granuloma? LHCH: involves the medullary cavities of bones, indolent (unifocal/multifocal)
Hand-Schuller-Christian Sydnrome? LHCH: calvarial defects, exophthalmos, diabetes insipidus (encroaches pit stalk); many spontaneously regress, can use chemo
You have a pt with a THYMOMA. What is the cell of origin? epithelial cell, not lymphoblasts
what is the most common site for extranodal involvement? stomach
B: leading cause of cancer deaths worldwide? gastric cancer
Created by: HSF2