Neoplastic Proliferations of White Cells Flashcards by Luke Cielonko

What viruses pose an increased risk for lymphoma

KSHV/HHV-8 = found in Kaposi sarcoma
EBV
Human T-Cell leukemia virus-1 (HTLV-1)

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What is the most common cancer in children?

Acute lymphoblastic leukemia/lymphoma (ALL)

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Positive TdT (terminal deoxynucleotidyl transferase) and Myeloperoxidase negative

Acute Lymphoblastic Leukemia/lymphoma

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Late B-ALL markers

CD10, CD19, CD20, and IgM Heavy chain

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Immature T-ALL markers

CD1, CD2, CD5, and CD7

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Factors that worsen the prognosis of ALLs

1) Under the age of 2
2) In adolescence or adulthood
3) Peripheral blood blast count greater than 100k
4) Presence of cytogentic aberrations (philadelphia chromosome)

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What is pathognomonic for chronic lymphocytic leukemia?

Proliferation centers of activated (mature) lymphocytes within the lymph nodes

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9:22; BCR-ABL

Philadelphia chromsome

1) Associated with worse prognosis in ALL
2) Found in chronic myeloid leukemia

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Most common cause of leukemia to adults in the western world; usually asymptomatic and has an indolent progression

Chronic lymphocytic leukemia (CLL)

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What peripheral precursor lymphoma is associated with BCL-2 involvement and arises from germinal centers

Follicular lymphoma

Hallmark: Translocation of IgH (14) with BCL2 (18)

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What molecular pathogenesis is involved with diffuse large B cell lymphoma?

Dysregulation of BCL6 (chromosome 3)

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Name the peripheral B cell neoplasms (8 total)

1) Chronic lymphocytic leukemia
2) Follicular lymphoma
3) Diffuse Large B cell Lymphoma
4) Burkitt Lymphoma
5) Plasma cell neoplasms
6) Mantle Cell Lymphoma
7) Marginal Zone Lymphomas
8) Hairy Cell Leukemia

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What disease has the pathognomonic finding on cytogenetics of a translocation of IgH (14) to BCL2 (18)

Follicular Lymphoma

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Subtypes of diffuse large B cell lymphoma assoc. with viruses

1) Primary effusion lymphoma- assoc. with HHV-8; presents with ascites or pleural effusion
2) Immunodeficiency-assoc. large B cell lymphoma assoc. with EBV; occurs with immunodeficiency of T cells (HIV and bone marrow transplant)

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Molecular pathogenesis found in all burkitt lymphoma

Translocation of c-MYC (8) to IgH locus (14)

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What is the etiology for Burkitt lymphoma

Generally occurs in children
Manifests at an extranodal site (endemic = mandible; sporadic - ileocecum and peritoneum)
Very Aggressive

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What system is generally affected in multiple myeloma?

Axial skeleton; findings include sharply, punched out bone lesions occurring most commonly in the vertebrae

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What are the most common findings of multiple myeloma?

1) Increased Ig (most common is IgG)
2) Bence-Jones protein in the urine
3) Renal dysfucntion
4) Pathologic fractures and chronic pain
5) Hypercalcemia (due to activation of osteoclasts via RANK receptor)
6) Recurrent bacterial infections (most common cause of death)

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What is the Rouleaux formation? What is it characteristic of?

The Rouleaux formation is a linear ray formation due to an increased amount of M proteins that cause cells to stick together found in Multiple myeloma

Found in other conditions with elevated Ig levels

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What is Waldenstrom macroglobulinemia?

It is a hyper viscosity state of blood that is due to high M protein levels; associated with lymphopasmacytic lymphoma; causes visual impairment, neurologic problems, and bleeding

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What is Monoclonal gammopathy of undetermined significance (MGUS)

This is an asymptomatic state with an M spike however it has no other findings of multiple myeloma; May be considered an early stage of multiple myeloma

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Lymphoma that is uncommon and resembles the mantel zone B cells that surround germinal centers; has high levels of cyclin D1 (increase progression from G1 to S phase)

Mantle cell lymphoma

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How do you differentiate mantle cell lymphoma from chronic lymphocytic lymphoma?

Mantle cell lymphoma = CD5+

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B- cell tumors that arise within lymph nodes, spleen, and extra-nodal tissues that have a memory B cell origin; arise within tissues involved with chronic inflammation

Marginal Zone Lymphomas

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What does Human T cell assoc. Leukemia Virus-1 cause?

Adult T cell leukemia (found only in CD4 T cells) | - Has clover cells; fatal within months

Tumors of CD4 helper T cells that accumulate in the skin

Sezary syndrome and Mycosis fungoides

What are the differences between sezary syndrome and Mycosis fungoides syndrome?

1) Mycosis fungoides - Occurs in stages (premycotic, plaque, tumor phases) 2) Sezary syndrome - Occurs as a generalized exfoliative erythroderma

What is the difference between a leukemia and a lymphoma?

Lymphoma - forms as a mass; nodal based origin (spleen, lymph nodes, thymus) Leukemia- neoplasm that presents with widespread involvement including the bone marrow

What are the differences with Hodgkin and Non-Hodgkin Lymphomas?

Hodgkin forms in nodal tissue and progresses very systematically Hodgkin is also characterized by the presence of Reed Sternberg cells

What is the progression of spread for Hodgkin lymphoma?

Lymph nodes --> Spleen --> Liver --> bone marrow --> extranodal

What cell is associated with Hodgkin lymphoma?

Reed Sternberg cells- release factors that suppress TH1 immune response

What are the classifications of Hodgkin lymphoma? (5 total)

1) Lymphocyte predominant 2) Mixed cellularity 3) Lymphocyte depletion 4) Nodular sclerosis (most common) 5) Lymphocyte rich

What Hodgkin lymphoma classifications have a similar immunophenotype for their Reed-Sternberg cells?

All except the lymphocyte predominant which has normal B cell phenotype (CD20 and BCL6); these are known as the classical forms (PAX5, CD15, CD30 positive; CD45 negative)

What is characteristic of Classic Reed Sternberg cells?

The way they die. Classic cells undergo a process called mummification where the cell shrinks and becomes pyknotic.

What class of Hodgkin lymphoma has lacunar Reed Sternberg cells, has collagen deposition, has the best prognosis, and is not associated with EBV?

Nodular sclerosis

What are the clinical features of Hodgkin lymphoma?

1) Painless lymphadenopathy 2) Cutaneous anergy (due to suppresive Th1 factors from Reed Sternberg cells) 3) Organized progression of disease

What is needed for the diagnosis of Acute Myeloid Leukemia?

The presence of at least 20% myeloid blasts in the bone marrow

What are the findings in the blood of a pt. with chronic myeloid leukemia?

Leukcytosis exceeding 100,000 cells | Increased basophils

What are the genetic abnormalities in acute myeloid leukemia and acute promyelocytic leukemia?

Mutations of tyrosine kinases (RARalpha 15:17) | RAR - retinoic acid receptor

Findings in bone marrow of pt. with chronic myeloid leukemia (Myeloproliferative disorder)?

1) Hypercellularity 2) Sea-blue Histiocytes (wrinkled green blue macrophages) 3) Increased deposition of reticulin

What is the mutation that is associated with polycythemia vera (PCV)?

The mutation to tyrosine kinase JAK2; upstream in regulation of growth factors involved with myeloid progenitor cells

What disease is associated with an increase in secretion of platelet derived growth factor and TGF-beta from megakaryocytes?

Primary myelofibrosis- causes an increase in fibroblast activity

What are the presenting clinical features of Primary myelofibrosis?

1) Dry tap 2) Teardrop-shaped red cells 3) Splenomegaly due to extramedullary hematopoieosis 4) Hyperurcemia (due to cell breakdown)

What pointmutations are associated with essential thrombocytosis?

JAK2 and MPL

What is associated with Birbeck granules? What are they?

Birbeck granules are pentalaminar tubules with a dilated terminal end producing a tennis racket appearance that are associated with Langerhans cell histiocytosis

What is a plasmacytoma?

A plasmacytoma is a solitary multiple myeloma lesion that will eventually progress into full multiple myeloma

What is an increased risk in the presence of Aeur rod?

Increases the incidence of DIC

Smudge cells are associated with what disease?

Chronic Lymphocytic leukemia

What is the chromosomal translocation of acute promyelocytic leukemia?

15;17- affects the RAR alpha gene

Treatement of promyelocytic leukemia?

ATRA; causes blasts to mature

Immunophenotype of chronic lymphocytic lymphoma?

CD5 and CD23

What do auer rods increase the risk for?

DIC (Disseminated Intravascular Coagulation)

What is characteristic of acute monoblastic leukemia?

Infiltration of the gums

What maker are hairy cells positive for?

TRAP

What are the complications of myeloprolifrative disorders

1) Hyperuricemia | 2) Bone marrow fibrosis leading to cytopenia

What are increased in chronic myeloid leukeima?

Basophils

What is the treatment of choice for pt. with chronic myeloid leukemia?

Imatinib; targets the tyrosine kinases which are altered due to translocation 9:22 (abl-bcr)

Where does the mutation of abl-bcr occur in chronic myeloid leukemia? Why is this important?

The mutation occurs in the pluripotent hematopoeitic stem cell. This is important because CML can transform into either AML or ALL.

What are the clinical findings of polycythemia vera?

1) Symptoms of hyperviscosity (blurred vision, increased risk of thrombosis, flushed face) 2) Increased risk of Budd-Chiarii

Disease that results in abnormalities of maturation within all myeloid lineages; High risk of transforming into acute myeloid leukemia; Causes peripheral blood cytopenia

Myelodysplastic syndrome

What disease is associated with peripheral blood cytopenias of every myeloid cell type and causes deformities as well?

Myelodysplastic syndrome

What are the myeloproliferative diseases? (4)

1) Chronic myeloid leukemia 2) Polycythemia vera 3) Essential thrombocytosis 4) Primary myelofibrosis

What is different in the presentation of a pt. with CML and AML in reference to the spleen?

``` AML= results in splenic rupture due to the aggressiveness of the disease CML= massive splenomegaly that does not burst due to its slow progression allowing for fibrosis to take place in the capsule ```

What can be generalized for all myeloproliferative diseases (CML, polycythemia vera, Essential thrombocytosis, and Primary myelofibrosis)

They all causes universal myeloid cytosis. Each specifically has more of their indicated cell.

What is an indicator of normal granulocytic cells?

Leukocyte alkaline phosphatase - High in leukemoid reaction - Low in CML

When is LAP high? Low?

``` High= leukemoid reaction Low= CML ```

What are the clinical findings of hairy cell leukemia?

1) Dry tap 2) Pancytopenia 3) Splenomegaly (invades red pulp) 4) No lymphadenopathy

How can acute myeloid leukemia occur?

1) De novo due to genetic alterations 2) From a chronic myeloproliferative disorder 3) From myelodysplastic syndrome

What are the levels of erythropoeitin found in polycythemia vera?

Low

What is the clinical progress of Follicular lymphoma?

1) Confined to lymph nodes and bone marrow 2) May transform into diffuse large B cell 3) Late peripheral blood spread 4) Indolent progression

What chronic inflammatory disorders are associated with marginal zone lymphoma?

1) Sjogren disease 2) Hashimoto thyroiditis 3) Helicobacter gastritis

Most common cause of a monoclonal gammopathy?

Monoclonal gammopahty of undetermined significance

Auer rod

Acute myeloid leukemia

What should you always look for in granulocytic cells?

Auer rods

Increased LAP (leukocyte alkaline phosphatase) you should think...

Leukemoid reaction

Pt presents with fever, night sweats, and weight loss. Cervical lymphadenopathy is noted on PE

Hodgkin lymphoma

Pt. presents with a fever and throat pain. Severe dyspnea, tachypnea, and inspiratory stridor. Labs show many immature hematopoietic cells. What is the cause?

T cell ALL 1) Thymic mass is common (results in dysphagia, dyspnea, and stridor) 2) Common in teens

Neoplastic Proliferations of White Cells Flashcards

(77 cards)