Term
| Morphology of Acute nonspecific lymphadenitis |
|
Definition
*Inflamed lymph nodes are:
Swollen
Grey-red
Engorged
Large germinal centers w. numerous mit figs
*Pyogenic infection:
Neutrophils about the node and in the sinuses
*Severe infection:
Central node necrosis
*General:
nodes are tender
overlying skin is red and penetration of infection may form draining sinuses.
depending on extent of inf. scar or no scar |
|
|
Term
| Morphology of Follicular hyperplasia |
|
Definition
Associated with B-cell activation
Follicular (germinal center) reaction
*Cells:
Activated B-cells
Macrophages containing nuclear debris (Tingible body macrophages)
Antigen presenting FDCs |
|
|
Term
| Causes of Follicular hyperplasia |
|
Definition
RA
Toxoplasmosis
Early HIV-infection |
|
|
Term
| Lymphadenitis can be confused with lymphomas. What findings favor diagnosis of lymphadenitis? |
|
Definition
1, Preserved lymph node architecture
2, Variation in nodule size and shape
3, Mixed population of lymphocytes at different stages of maturation
4, Prominant phagocytic and mitotic activity in germinal center |
|
|
Term
| Morphology of Paracortical Hyperplasia |
|
Definition
Reactive changes in T-cell regions of the lymph nodes.
Parafollicular T-cells transform into large immunoblasts upon activation |
|
|
Term
| Causes of Paracortical Hyperplasia |
|
Definition
Viral infection (EBV)
Following certain Vaccinations (smallpox)
Immune reactions to certain drugs (phenytoin) |
|
|
Term
| Morphology of Sinus histiocytosis |
|
Definition
Distention and prominence of lymph sinusoids
Marked hypertrophy of endothelium
Infiltrate of macrophages (Histiocytes) |
|
|
Term
| Causes of Sinus histiocytosis |
|
Definition
Often found in lymph nodes draining cancers.
May represent immune response to the tumor or its product |
|
|
Term
| Diseases of LYMPHOID NEOPLASMS and their cell normal differentiation stage |
|
Definition
NHLs
Hodgkin lymphomas
lymphocytic leukemias
plasma cell dyscrasias
*Normal cell differentiation |
|
|
Term
| Subcategories of MYELOID NEOPLASMS, their characteristics,cells involved |
|
Definition
Acute myeloid leukemias (immature in BM and PB)
Chronic myeloproliferative disorders (increased blood cell count)
Myelodysplastic disorders (ineffective hematopoiesis and cytopenias)
Cells: RBCs, Granulocytes, platelets |
|
|
Term
| What is the name of plasma cell tumors, where are they situated and what are the systemic signs of such a tumor? |
|
Definition
Plasma cell dyscrasia.
Found as discrete masses in bone.
Produces complete or incomplete monoclonal antibodies |
|
|
Term
| Which is the most common immune cell type associated with adult lymphomas, whats their differation stage and from where do they originate? |
|
Definition
| Mature follicular or post follicular B-cells that have undergone somatic hypermutation as well as isotype switch. |
|
|
Term
| Whats the clonality of lymphomas? |
|
Definition
|
|
Term
| Whats the normal spreading fashion of Hodgkin lymphomas |
|
Definition
| Normally present at a single site and spreads in a predictable fashion to contiguous lymph nodes. |
|
|
Term
| The WHO-classification, on what basis does it classify lymphoid neoplasm and which categories are considered? |
|
Definition
Morphology, cell of origin (immunophenotyping), clinical features and genotype (karyotype, viral genome).
B-cell tumors, T-and NK cell tumors, Hodgkin lymphomas |
|
|
Term
| What is the common name for B- and T-cell lymphoblastic lymphoma/leukemia? |
|
Definition
| Acute lymphoblastic leukemia, ALL |
|
|
Term
| Which age group is normally assiciated with ALL? |
|
Definition
| children and young adults |
|
|
Term
| What is the normal origin of B- and T- lyphoblastic tumors? |
|
Definition
Pre B-cell: Bone marrow -> leukemia
pre T-cell: Thymus -> leukemia |
|
|
Term
| General pathophysiological features of acute leukemias |
|
Definition
Rapid growing tumor (normal BM progenitors grow faster!)
Block in differentiation
Bone marrow failure in time
Accumulation of immature blast cells ->
Supression of normal hematopoiesis by PHYSICAL DISPLACEMENT |
|
|
Term
| Clinical features of acute leukemias |
|
Definition
Abrupt stormy onset
BM depression: fatigue (anemia), fever (infections), bleeding (gum bleeding, petichiae etc) 2nd to thrombocytopenia
Bone pain (BM expansion)
Generalized lymphadenopathy, spleno and hepatomegaly (more in ALL than in AML)
CNS (headache, vomiting, nerve palsies) |
|
|
Term
| Laboratory findings in ACUTE LEUKEMIAS |
|
Definition
Identification depends on blast cell identification in PB and BM.
White cell count: sometimes elevated to 100,000/ul but in 50% less than 10,000/ul.
Anemia
Platelet count < 100,000/ul
Neutropenia |
|
|
Term
|
Definition
25% of BM cellularity are blast cells
Nuclei show clumped chromatin
One-two nucleoli
Cytoplasm is rich in PAS+ material |
|
|
Term
|
Definition
Finer chromatin than ALL
More cytoplasm with granules
Myeloblasts are normally peroxidase positive |
|
|
Term
| Give 7 examples of precursor B-cell neoplasms according to WHO |
|
Definition
1. CLL B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma
2. Mantle cell lymphoma
3. Follicular lymphoma
4. Extranodal marginal zone (malt) lymphoma
5. Plasma cell myeloma
6. Diffuse large B-cell lymphoma
7. Burkitt lymphoma |
|
|
Term
| Give 5 examples of precursor T-cell neoplasms according to WHO |
|
Definition
1. T-cell prolymphocytic leukemia
2. Mycosis Fungoides/Sezary syndrome
3. Peripheral T-cell lymphoma (NOS)
4. Adult T-cell lymphoma/leukemia (HTLV1)
5. NK cell leukemia |
|
|
Term
| Give 5 examples of Hodgkin lymphomas according to WHO |
|
Definition
1.Lymphocyte predominans, nodular
2. Nodular sclerosis
3. Mixed cellularity
4. lymphocyte rich
5. Lymphocyte depletion |
|
|
Term
| Immunophenotyping findings and differentiation of ALL and AML |
|
Definition
Terminal deoxytransferase (TdT) found in 95% of all pre B- and T-cells
B-cells CD19+
T-cells CD3+ |
|
|
Term
| Karyotypic changes in pre B-cell tumors |
|
Definition
90% of patients have nonrandom karyotypic abnormalities.
*Pre B-cell tumors: hyperploidity (>50N)
t(12;21) involving TEL1 and AML1 genes
Poor outcome in translocations of MLL gene on 11q23 or t(8;22) philadelphia |
|
|
Term
|
Definition
| Receptor for T-cell essential for T-cell development. It promotes T-cell survival and proliferation. Makes it possible for stem cells to differentiate into T-cell outside the thymus. |
|
|
Term
|
Definition
| 55-60% of pre T-cell tumors show point mutations in NOTCH1. Explains why patients with pre T-cell tumors have BM and not thymus involvement. |
|
|
Term
| Prognosis of Lymphoblastic tumors |
|
Definition
The prognosis for children 2-10 yrs have the best prognosis. Above and below this range do less well.
Karyotypic changes such as MLL and philadelphia chromosome is common UNDER the age of 2 and in adults.
Karyotypic changes at 2-10 yrs include t(12;21) |
|
|
Term
| Similarities and differences btw CLL and SLL |
|
Definition
Similarities:
morphology, phenotype and genotype are identical
Differences: peripheral blood involvement.
CLL: >4000/ul
SLL: <4000/ul |
|
|
Term
| Clinical and pathophysiology of SLL/CLL |
|
Definition
Clinical: Hypogammaglobulinemia, autoantibodies against RBCs
with time displacement of healthy BM leading to Anemia, neutropenia and thrombocytopenia.
Pathophys: Autoantibodies are produced by non tumor B-cells indicating a general breakdown of immune regulation. |
|
|
Term
|
Definition
Sheets of small round lymphocytes predominate and scattered foci of larger dividing lymphocytes (proliferation centers) efface involved lymph nodes.
Mitotic figures are rare.
Lymph nodes, BM, spleen and liver are almost always involved.
Smudge cells are characteristic (fragile neoplastic lymphocytes) |
|
|
Term
|
Definition
| SLL/CLL is a neoplasm of mature B-cells and express CD5, CD19, CD20 and CD23 as well as surface immunoglobulin heavy and light chains. |
|
|
Term
|
Definition
50% of patients show karyotypic abnormalities. most common is trisomy 12 and deletions of 11 and 12.
Translocations are rare.
Most SLL/CLLs have undergone somatic hypermutation. |
|
|
Term
| Clinical findings of SLL/CLL |
|
Definition
SLL/CLL are often asyptomatic at presentation. Nonspecific symptoms include: fatigue, weight loss and anorexia.
Generalized hepatosplenomegaly and lymphadenopathy in 50-60%
Cell count may increase slightly (SLL) or may reach 200,000/ul.
50% hypogammaglobulinemia |
|
|
Term
|
Definition
Median survival is 4-6 yrs. With time, SLL/CLL tend to transform into more agressive tumors, either pro-lymphocytic leukemia or diffuse large B-cell lymphoma.
Once transformed, median survivial is one year. |
|
|
Term
| According to WHO, how is follicular lymphoma classified? |
|
Definition
| Peripheral B-cell neoplasm. Non hodgkin lymphoma |
|
|
Term
| Morphology of follicular lymphoma |
|
Definition
Lymph node proliferations have a NODULAR APPEARANCE.
the predominant neoplastic cell is CENTROCYTE-LIKE (small) with prominant nuclear indentations indistict nucleolus mixed with larg centroblast-like cells.
Infrequent Mitotic figures. |
|
|
Term
| Markers of Follicular lymphoma |
|
Definition
CD10, CD19,CD20 AND BCL 6 (transcription factor req for follicular center formation).
BCL 2 (not expressed by normal follicular B-cells)
Immunoglobulin genes have undergone somatic hypermutation. |
|
|
Term
| Karyotype of Follicular lymphoma |
|
Definition
| The majority of tumors have t(14;18), fusing the BCL2 gene on 18q21 with IgH gene on 14 -> overexpression of BCL2 protein preventing apoptosis |
|
|
Term
| Clinical features of Follicular lymphoma |
|
Definition
Mostly in older people
Equal in male and female
PAINLESS GENERALIZED ADENOPATHY
BM almost always contain lymphoma
40% progress into diffuse large B-cell lymphoma |
|
|
Term
| Cells and location of Mantle cell lymphomas |
|
Definition
| B-cells that resemble the cells of the mantle zone of normal lymph follicles |
|
|
Term
| Morphology of Mantle cell lymphomas |
|
Definition
Lymph nodes are involved in a diffusely nodular pattern. Cells are slightly larger than its healthy counterpart with an irregular nucleus and no visible nucleolus.
BM is frequently involved. 20%of pats have PB involvement.
GI-tract is frequently involved, sometimes in the form of multifocal submucosal nodules that grossly resemble POLYPS |
|
|
Term
| Immunophenotyping of Mantle cell lymphomas (markers) |
|
Definition
Coexpression of IgM and IgD
CD5, CD19 AND CD20
To differentiate from SLL/CLL, MCLs express CYCLIN D1 as well as the absence of proliferation centers. |
|
|
Term
| Karyotypic changes in Mantle cell lymphomas |
|
Definition
most tumors have t(11;14) that fuses the cyclin D gene on 11 with IgH locus on 14.
The immunoglobulin genes have NOT undergone somatic hypermutation proving the origin of NAIVE B-CELLS |
|
|
Term
| Clinical features of Mantle cell lymphomas |
|
Definition
Fatigue and lymphadenopathy
Generalized disease in spleen, BM, liver and often GI-TRACT (POLYPS) |
|
|
Term
| Which age group is mostly affected by Diffuse large B-cell lymphoma and how frequent is it? |
|
Definition
| Most important ADULT type of lymphoma. It accounts for 50% of adult NHLs |
|
|
Term
| Morphology of Diffuse large B-cell lymphoma |
|
Definition
Nuclei of neoplastic cells are 3-4 times the size of resting lymphocytes.
Cells resemble CENTROBLASTS.
Variable shaped nuclei with several distinct nucleoli.
In other tumors immunoblast-like cells are found. They have abundant cytoplasm and resemble cells normally found in the paracortex of lymph nodes. |
|
|
Term
| Immunophenotype and molecular markers of Diffuse large B-cell lymphomas |
|
Definition
MATURE B-CELL TUMOR: pan-B-cell antigens, CD19 and CD20.
Many express IgM and/or IgG
CD10 occationally
Cells havve undergone somatic hypermutation |
|
|
Term
| Karyotype of Diffuse large B-cell lymphomas |
|
Definition
30% have t(14;18) involving the BCL2 gene (may represent transformed lymphmas.
30% have rearrangements in BCL6, but even more frequently have mutations in BCL6.
Both alterations show increased synthesis of BCL6 |
|
|
Term
| Subtypes of Diffuse large B-cell lymphomas |
|
Definition
EBV is implicated in diffuse large B-cell lymphomas related to AIDS and transplant patients.
HHV-8 (Kaposi) is associated with PRIMARY EFFUSION LYMPHOMAS within the serosal membranes.
Mediastinal large B-cell lymphomas are usually present in young females. they spread predominantly to the abdominal viscera and CNS |
|
|
Term
| Clinical features of Diffuse large B-cell lymphomas |
|
Definition
Rapidly growing, agressive, often symptomatic, masses at one or several sites.
Fatal if not treated.
Extranodal masses are common, brain, GI are most common. Though growths can arise in any organ.
Liver, spleen and BM are not common. |
|
|
Term
| Morphology of Burkitt Lymphoma |
|
Definition
B-cells are intermediate in size (macrophage).
Nucleus is round or oval with 2-5 PROMINANT NUCLEOLI
Cytoplasm contain small lipid-filled vacuoles.
High mitotic rate and cell death is characteristic.
Numerous macrophages are present and a clear space surrounds them, STARRY SKY pattern |
|
|
Term
| Immunophenotype and molecular markers of Burkitt Lymphoma |
|
Definition
B-cells express:
surface IgM, kappa or lambda chains
CD10, CD19, CD20 pan-B-cell markers
Immunoglobulin genes have undergone somatic hypermutation. |
|
|
Term
| Karyotype changes in Burkitt Lymphoma |
|
Definition
| B-cells are always associated t(8;14) involving MYC gene on 8 fused with IgH on 14 resulting in dysregulation and overexpression of MYC protein. |
|
|
Term
| Clinical features of Burkitt Lymphoma |
|
Definition
Mainly children and young adults.
Tumors normally develop extra nodally; mandible, maxilla, bowels, retroperitoneum, and ovaries.
Leukemia is uncommon
Burkitt lymphoma is one of the fastest growing human neoplasms.
With aggressive treatment, majority can be cured |
|
|
Term
| Clonality of plasma cell dyscrasias and secretory product |
|
Definition
Monoclonal
Secretes complete or partial immunoglobulins |
|
|
Term
| Six categories of plasma cell dyscrasias |
|
Definition
1, Multiple myeloma
2, Localized plasmacytoma (solitary myeloma)
3, Lymphoplasmacytic lymphoma
4, Heavy chain disease
5, primary or immune associated amyloidosis
6, Monoclonal gammaopathy of undetermined significance |
|
|
Term
Multiple myeloma:
1, Cells
2, Location
3, Karyotypic changes
4, Secretory product
5, Clinical findings |
|
Definition
1, Neoplastic plasma cells
2, Bonemarrow
3, Chromosomal translocations involving IgH on 14 fused with cyclin D1 and D3, FGF-R3. Sometimes MYC is also involved.
4, M-components (paraprotein): IgG 60%, IgA 20-25%, rarely IgM, IgD and IgE, 15-20% light chains known as Bence-Jones when found in the urine.
5, Impaired renal function |
|
|
Term
Localized plasmacytoma
1, Cells
2, Location
3, Secretory product
4, Other characteristics |
|
Definition
1, Neoplastic plasmacells
2, Solitary lesions in bone or soft tissue (upper respiratory tract)
3, Modestly elevated paraproteins in some patients.
4, Solitary skeletal plasmacytomas usually have hidden disease elsewhere and most develop multiple myeloma in 5-10yrs |
|
|
Term
Lymphoplasmacytic lymphoma
1, Cells
2, Location
3, Secretory product
4, Other characteristics |
|
Definition
1, Mixed proliferation of B-cells, small lymphocytes to plasma cells
2, Commonly multiple lymph nodes, BM and spleen
3, Paraprotein: unlike Multiple myeloma, IgM is the most common -> increased viscosity of blood and Waldenströms macroglobulinemia.
4, Balance btw heavy and light chain secretion prevents Bence-Jones proteins in the urine. No lytic bone lesions |
|
|
Term
Heavy chain disease
1, Cells
2, Location
3, Secretory products |
|
Definition
1, Plasma cells
2, IgA heavy chain disease is mostly found in MALT (small intestine and resp. tract)
3, Only heavy chains are secreted |
|
|
Term
Monoclonal gammopathy of undetermined significance
1. Cells
2,Characteristics |
|
Definition
1, Plasma cells
2, Most common plasma cell dyscrasia, 1-3% of people over 50.
Precursor lesion concidered a neoplasia as 1% develop plasma cell dyscrasia at 1%/yr.
Often the same translocations found in multiple myeloma are present (IgH on 14 with Cyclin D1,3)
<30gr/l monoclonal protein and NO BENCE-JONES PROTEINURIA |
|
|
Term
| Morphology of Multiple myeloma |
|
Definition
Multifocal destructive bone lesions. in decreasing freq: vertebral column, ribs, skull etc
Pathologic fractures mostly in the VC
10-90% of BM cellularity is normal and abnormal plasma cells
Later, spleen, liver, kidneys and lungs are involved and eventually leukemia presents.
Renal involvement (myeloma nephrosis)with proteins present in distal and collecting ducts. B-J and amyloid casts are surrounded by multinucleated macrophages and necrotic endothelium adjacent to cast. |
|
|
Term
| Clinical course of plasma cell dyscrasias |
|
Definition
In Multiple myeloma the clinical signs are from plasma cells tumors, while in lymphoplasmacytic lymphoma symptoms result from IgM in the serum.
Bone pain is very common.
Hypercalcemia -> neurologic manifestations
Recurrent infections from supressed immune function
Hyperviscosity syndrome from myeloma proteins
Renal insufficiency in 50% of pats (B-J proteins)
Amyloidosis 10-15% of pats
Etc. see page 456 in Robbins |
|
|
Term
Hodgkin Lymphoma
1, Distinctive cells
2, Location spreading
3, Tumor origin |
|
Definition
1, Neoplastic Reed-Sternberg giant cells
2, Single lymph node and stepwise spreading to draining lymph nodes
3, B-cell |
|
|
Term
| Classify the five forms of Hodgkin lymphoma |
|
Definition
1, Nodular sclerosis
2, Mixed cellularity
3, Lymphocyte predominant
4, Lymphocyte rich
5, Lymphocyte depletion |
|
|
Term
| Morphology of Nodular sclerosis (HL) |
|
Definition
Most common form.
Most often found in lower cervical, mediastinal and supraclavicular lymph nodes.
Excellent prognosis
LACUNAR CELLS, large multilobe nucleus with multiple nucleoli and abundant pale cytoplasm.
Collagen bands divide the lymph node into nodules.
INFREQUENT RS-CELLS |
|
|
Term
| Immunophenotype and markers of nodular sclerotis HL |
|
Definition
Lacunar cells are identical to RS-cells.
CD15 and CD30, usually no B-cell markers |
|
|
Term
| Morphology of Mixed-cellularity HL |
|
Definition
Most common HL after 50yrs, 25% of total
Male predominance
NUMEROUS RS-CELLS
Heterogenous cellular infiltrate (plasma cells, small lymphocytes, eosinophils and benign histiocytes)
Systemic symptoms of dissiminated disease is common |
|
|
Term
| Morphology of Lymphocyte predominance HL |
|
Definition
5% of all HLs
Large number of small resting lymphocytes
variable number of benign histiocytes
RS-CELLS ARE VERY RARE, but when found they are called POPCORN CELLS
Follicular B-cell origin (CD20+) w. somatic hypermutation
Cervical or axillary solitarty lymphadenopathy
Excellent prognosis |
|
|
Term
| Molecular changes in Hodgkin Lymphoma |
|
Definition
EBV genome is present in 70% of mixed cellularity type and a smaller fraction in nodular sclerosis.
EBV genome is identical in all RS-cells, indicating preceding infection.
High levels of NF-kB
RS-cells release IL-5, IL-13 and TGF-B which attract non-neoplastic cells which in turn activate RS-cells (CD30L) |
|
|
Term
| Important translocation in AML, its fusion product, and its significance |
|
Definition
t(15;17)
retinoic acid receptor alpha (17) with PML (15).
In AML with this translocation, therapeutic doses of vitamin A may induce maturation of the blast into a mature neutrophil, that dies within 6 hours. |
|
|
Term
| FAB classification of AML M0-M3 and their characteristics |
|
Definition
M0: Minimally dif. AML. blasts lack Auer rods and MPO. linage surface markers
M1: AML without maturation. >3% are MPO+, few auer rods and granules
M2: AML w. maturation: >20% are blasts and more differentiated. Auer rods. t(8;14). Most common AML
M3: A. promyelocytic leu. Many Auer rods/cell, DIC, t(15;17) |
|
|
Term
| FAB classification of M4 and its characteristics |
|
Definition
| M4: A. Myelomonocytic leu. myelo and monocytic dif by cytochem stains. Monoblasts NSE+ |
|
|
Term
| FAB classification of M5 and its characteristics |
|
Definition
M5: A. monocytic leu. blasts and immature cytes. MPO-,NSE+. No auer rods. organomegaly.
M5a: only immature cells
M5b: mature cells in the peripheral blood |
|
|
Term
| FAB classification of M6 and its characteristics |
|
Definition
| M6: A. erythroleukemia. Abundant dysplastic erythroid progenitors. >20% are non erythroid myeloblasts with auer rods. |
|
|
Term
| FAB classification of M7 and its characteristics |
|
Definition
| M7: A. megakaryocytic leu. Blasts of megakar. linage predominate by PLT specific antigen. myelofibrosis or reticulin+ common. NO AUER RODS |
|
|
Term
|
Definition
MPO+, especially auer rods, which are MPO++.
Monocytic differentiation: NSE |
|
|
Term
|
Definition
Most express: CD 13, 14, 15, 64 or 117 (cKIT).
CD 33 is important in distiguishing AML from ALL as well as identifying M0.
Monoclonal antibodies identify M7 |
|
|
Term
| General bone marrow characterstics of myelodysplastic syndromes |
|
Definition
| BM is partly or wholly replaced by clonal progenitors of multi pot SC. it can dif into all myeloid linages, but is INEFFECTIVE. BM is normo or hypercellular. SC is unstable and aquires additional mutations. May transform into AML. most cases idiopathic but some after radiation or chemotherapy |
|
|
Term
| Karyotypic changes is myelodysplastic syndromes |
|
Definition
| Common loss of chromosome 5 or 7 or 5q or 7q. |
|
|
Term
| General features of chronic myeloproliferative disorders |
|
Definition
Hyperproliferation of neoplastic myeloid progenitors, with the ability of terminal differentiation.
Peripheral blood show increase in one or more formed elements.
Organ seeding of 2nnd hematopoietic organs -> hepatosplenomegaly and mild lymphadenopathy.
Common tyrosine kinase mutations. |
|
|
Term
| Name the four subcategories of chronic myeloproliferative disorders. |
|
Definition
1. Chronic myelogenous leukemia
2. Polycythemia vera
3. Essential thrombocythemia
4. Primary myelofibrosis. |
|
|
Term
| Characteristic translocation of CML |
|
Definition
| Philadelphia chromosome, t(9;22), abl/bcr |
|
|
Term
| The most common mutation in P Vera, myelofibrosis, and ET |
|
Definition
A mutation in JAK2 kinase:
>90% of PV
50% in myelofibrosis
30% of essential thrombocythemia |
|
|
Term
|
Definition
Leukocyte count elevated, often 100.000+/ul.
Circulating cells are: neutrophils, metamyelocytes and myelocytes. <5% of PB are blasts.
Thrombocytosis is typical.
Hypercellular BM.
Red pulp of spln resembles BM->splenic infarcts |
|
|
Term
|
Definition
Chronic phase: slow progression. without treatment, median survival is 3 yrs.
Accelerated phase: 50% enter this phase. gradual loss of response to treatment, increasing anemia and thrombocytopenia.
Blast crisis: remaining 50% enter abruptly without accelerated phase.
30% show pre-B-cell morphology.
70% resembles AML |
|
|
Term
|
Definition
Bone marrow transplantation is curative in 70%
Inhibition of BCR-ABL tyrosine kinase |
|
|
Term
| General characteristics of Pylocythemia vera, PCV |
|
Definition
Excessive neoplastic proliferation and maturation of erythoid, granulocytic and megakaryocytic elements -> PANMYELOSIS.
The most striking change is the absolute increase in red cell mass, with low EPO. |
|
|
Term
|
Definition
Increased blood volume and viscosity -> thrombosis in heart, spleen and kidneys. Congestion of organs.
Hepatic enlargement with foci of hematopoiesis.
Distention of blood vessels -> hemorrhage
Some degree of BM fibrosis in 10% |
|
|
Term
|
Definition
Late middle age
Histamine release -> intense itching and peptic ulcers.
Hypertension -> headache, dizzy, hematemesis, melaena.
Cell turnover+ -> gout and asympt. hyperuricemia. |
|
|
Term
| RBC count and hematocrit of PCV |
|
Definition
6-10T/L
hematocrit: around 60% |
|
|
Term
|
Definition
After prolonged survival with treatment PCV tends to evolve a post polycythemic state, characterized by primary myelofibrosis.
Shift of hematopoiesis to the spleen.
Transformation to AML is possible. |
|
|
Term
| General features of primary myelofibrosis. |
|
Definition
Extreme hepatosplenomegaly as a concequence of BM fibrosis.
Non neoplastic FBs are stimulated by PDGF and TGF-beta from neoplastic megakaryocytes. |
|
|
Term
| Morphology of primary myelofibrosis |
|
Definition
Principle site of extramedullary hematopoiesis is the SPLEEN (up 4kg), with subcapsular infarcts.
Hypocellular BM, diffuce fibrosis
Myeloid precursors are bizzare in morph.
Moderate hepatomegaly and lymph node enlargement, with HP foci. |
|
|
