Chapter 13 Flashcards by Garrison Starrett

Hodgkin lymphoma subtype:
Nonclassical subtype
Excellent prognosis
Express BCL6 (unique to this subtype)
Not associated with EBV
“Popcorn cells”

Lymphocyte-predominance subtype

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Langerhans cell histiocytosis subtype:
2 year old boy has had a seborrheic eruption over the scalp and trunk over the past month. He then develops a right ear ache. On physical exam, the right tympanic membrane is erythematous and bulging. He has hepatosplenomegaly and generalized lymphadenopathy. Lab studies show Hgb 9.5 g/dL, Hct 28.7%, MCV 90 fL, platelet count 58,000/microliter, and WBC count 3540/microliter. Bone marrow biopsy shows 100% cellularity with extensive infiltration by cells resembling macrophages that express CD1a antigen and, by electron microscopy, have prominent HX bodies (Birbeck granules).

Letterer-Siwe disease and localized eosinophilic granuloma

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Hodgkin lymphoma subtype:
Uncommon
Reactive lymphocytes make up majority of cellular infiltrate
Lymph nodes diffusely effaced
Very good to excellent prognosis

Lymphocyte-rich subtype

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AML class I (genetic aberrations) favorable vs poor prognosis

Favorable: t(8:21) and inv(16)
Poor: t(11:q23:v)

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When a high level of M proteins causes RBCs in blood smears to stick together linearly
Characteristic of multiple myeloma

Rouleaux formation

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HIV associated Burkitt lymphoma has __% of patients latently infected with EBV

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Is CMML (chronic myelomonocytic leukemia) a myelodysplastic syndrome or a myeloproliferative disorder?

Myelodysplastic syndrome (MDS)

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Thymocytes hypoplasia/aplasia:
What is the name of this syndrome?
Which pharyngeal pouches is there a problem with developing?

DiGeorge Syndrome; 3rd and 4th pharyngeal pouches

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Immunophenotype (CD markers) of CLL

CD 19, 20, 23

ALSO CD 5 - B cell proliferation that is positive for CD 5 = CLL

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Pentalaminar tubules with a dilated terminal end (“tennis racket” appearance)
What pathology are they seen in?

Birbeck granules

Seen in langerhans cell histiocytosis (Birbeck granules contain langerin)

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Tumors (2) that present with CLA (+), CCR4 (+) and CCR10 (+)

Mycosis fungoides and Sezary syndrome

CLA = cutaneous leukocyte antigen

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Immunophenotype of langerhans cell histiocytosis

HLA-DR (+)
S100 (+)
CD1a (+)

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Multiple Myeloma:
Bence-jones proteinuria:
Excreted _____ chains are toxic to renal tubular epithelial cells -> kidney myeloma -> renal failure/insufficiency (second MCC of death in MM)

Light

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Lymphoma with t(11:14) cyclin D1/IgH

Overexpression of cyclin D1 promotes G1->S phase progression

Mantle Cell Lymphoma

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Leukemia/lymphoma with the following clinical features:
Massive splenomegaly
Exceptionally sensitive to genital chemotherapy -> long lasting remission
Excellent prognosis

Hairy cell leukemia

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Leukemia/lymphoma with large anaplastic cells with horseshoe nuclei and voluminous cytoplasm as hallmark cells

Anaplastic large cell lymphoma (ALK+)

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Hodgkin lymphoma subtype:
Most common form
Adolescent/young adults
Excellent prognosis
Lacunar cells
Deposition of collagen bands that divide lymph nodes into circumscribed nodules

Nodular sclerosis subtype

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Thymomas typically contain _____ (cell)

Thymocytes - immature T cells

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Leukemia/lymphoma with the following features:
Destructive nasopharyngeal mass
More common in Asia
Tumor originates from a single EBV-infected cell
Small to large pythonic cells cuffing and invading small vessels with ischemic necrosis
CD21 (-) and CD3 (-)

Extranodal NK/T-cell lymphoma

Extra-nodal = extra-“nosal”

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Langerhans cell histiocytosis subtype:
Benign
Adolescents
Pathologic fracture
Skin is NOT involved

Eosinophilic granuloma

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Most common non-random chromosomal abnormality seen in WBC neoplasms

Translocations

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Most common plasma cell disorder/dyscrasia

most common plasma cell neoplasm/malignancy is multiple myeloma

Monoclonal Gammopathy of Uncertain Significance (MGUS)

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Splenomegaly can cause hypersplenism characterized by ___, ____, and ____ either alone or in combination

Anemia, leukopenia, and thrombocytopenia

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Bence-Jones proteins are light chains small in size that are excreted in the urine and are seen in _______

Plasma cell neoplasms (dyscrasias)

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BCL6 is required for formation of normal germinal centers. What leukemia/lymphoma results from a dysregulation of BCL6?

DLBCL

AML class III (therapy related) favorable vs poor prognosis

Only very poor prognosis

``` Langerhans histiocytosis subtype: Malignant Young children (>3 years old) Triad of calvarial (skullcap) bone defects, diabetes insipidus, and exophthalmos Scalp rash ```

Hand-Schuller-Christian disease

How do you differentiate between benign and malignant thymomas? What are the 3 histologic subtypes of thymomas?

Cellular atypia; Thymoma - cytologically benign and noninvasive Invasive thymoma (type I malignant thymoma) - cytologically benign but locally invasive/metastatic (no cellular atypia) Malignant thymoma (thymic carcinoma, type II malignant thymoma) - cytologically malignant (cellular atypia)

DLBCL subtype always associated with Kaposi’s | Tumor cells infected with KSHV/HHV8 appears to have a causal role

Primary Effusion Lymphoma

Leukemia/lymphoma presenting with BRAF (serine/threonine kinase) activating mutation (valine -> glutamate)

Hairy cell leukemia

Lymphoma characterized by an acquired MYD88 mutation

Lymphoplasmacytic lymphoma

Burkitt lymphoma subtype - ALL patients latently infected with EBV Involves mandible/face

African (endemic)

Mechanism of pancytosis (high counts of all cells) in myeloproliferative disorders

Overactive (mutation) tyrosine kinase causing growth factor independence

Prognosis of Adult T-cell Luekemia/lymphoma (ATLL)

Rapidly progressive disease | Fatal in months-1 year even with aggressive chemotherapy

T/F - fever presents in Hodgkin lymphoma

TRUE!

Leukemia/lymphoma with the following: HUGE spleen Low LAP Basophilic + eosinophilia

Chronic myeloid leukemia (CML)

Factors influence WBC neoplasia: | Where do oncogenic mutations occur most frequently in during attempted antibody diversification?

Germinal center B cells

Monoblasts/monocytes are positive for _________

Non-specific esterase

Which lymphoma spreads in an orderly fashion and staging is useful

Hodgkin’s | Has distinct pathologic features and is treated in a unique fashion

Myeloma with elevated serum M protein >3 g/dl but ASYMPTOMATIC

Smoldering myeloma

Most common leukemia of adults in Western world

CLL

Neoplasm that presents with lytic bone lesions, hypercalcemia, renal failure, and acquired immune problems

Multiple Myeloma

Which leukemia/lymphoma has a waxing-waning course?

Follicular lymphoma

DLBCL subtype that occurs with severe T-cell immunodeficiency (HIV/allogenic bone marrow transplant)

Immunodeficiency associated Large B-cell Lymphoma

Kids with ______ have ALL until proven otherwise

A very low platelet count

Are CNS symptoms such as nerve palsies seen in ALL or AML?

ALL | No nerve palsies seen in AML

Are patients with polycythemia Vera prone to thrombosis or bleeding?

Both Elevated hct -> increased blood viscosity + thrombocytosis and abnormal platelet function -> prone to both thrombosis and bleeding

``` Hodgkin lymphoma subtype: Second most common type Male predominance Biphasic age distribution Prognosis very good ```

Mixed cellularity subtype

Burkett lymphoma immunophenotype

Immunophenoytpe = mature B cells CD 10, 19 20 (+), BCL6 (+), surface IgM (+) MYC (+) *BCL2 (-)

Myelodysplasias are characterized by ______

Thrombocytopenia

t(11:18), t(14:18) or t(1:14) are specific for ___________

Extranodal marginal zone lymphomas (MALTomas)

AML class II (MDS-like features, i.e. myelodysplastic syndrome) favorable vs poor prognosis

Only poor prognosis

Thymic epithelial cells are _____ cells

Squamous

Myeloproliferative disorder with valine -> phenylalanine at residue 617

Polycythemia Vera

Most common form of non-Hodgkin’s lymphoma

Diffuse Large B-cell Lymphoma (DLBCL) | Poor prognosis

``` Langerhans cell histiocytosis subtype: Malignant Infant (<2 years old) Skin rash (trunk and scalp) and cystic skeletal defects Rapidly fatal ```

Letterer-Siwe disease | Remember 2 names = <2 years old

Lymphoma with a substantial fraction of tumor cells that undergo terminal differentiation to plasma cells (distinctive factor from CLL)

Lymphoplasmacytic lymphoma

What tumor commonly arises in Waldeyer’s Ring (oropharyngeal lymphoid tissue including tonsils AND adenoids)

DLBCL

``` Myelodysplastic syndromes (MDS) can be characterized by a pathogenesis that includes: Monosomies 5 and 7 Deletions of 5q, 7q, and 20q Trisomy 8 Which chromosome is the MYC gene on? ```

Chromosome 8

Prognosis of anaplastic large cell lymphoma (ALK+)

Good prognosis

Syndrome characterized by RA, splenomegaly, and neutropenia | Caused by large granular lymphocytic leukemia

Felty syndrome

Favorable prognoses for ALL

``` Hyper-diploidy Age between 2 and 10 Low white cell count Trisomy of chr 4, 7, or 10 Presence of t(12:21) - involves ETV6 and RUNX1 genes ```

Most common plasma cell neoplasm

Multiple Myeloma

The spent phase of myeloproliferative disorders is only seen in ______ Looks like primary myelofibrosis

Polycythemia vera

B-cell tumors present with _____ tumors

Cyclic

Follicular lymphoma chromosomal associations

Strongly associated with chromosomal translocations involving BCL2 t(14:18) BCL2/Ig heavy chain - seen in almost all follicular lymphomas IGH locus on chromosome 14 BCL 2 locus on chromosome 18 BCL2 antagonizes apoptosis (promotes cell survival, devoid of apoptotic cells because BCL2 is anti-apoptotic)

CLL effect on immune function

Hypogammaglobulinemia = bacterial infection

Hodgkin lymphoma - EBV vs non-EBV associated types

``` Classical types (nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted) - associated with EBV infection Lymphocyte-predominant is NOT associated with EBV infection ```

Which myeloproliferative disorder are dacrocytes seen in (and what are they)?

Primary myelofibrosis | Dacrocytes (“daCRYocytes”) - tear drop shaped cells (damaged during release form fibrotic marrow)

Lymphoplasmacytic lymphoma-related syndrome in which high levels of IgM leads to symptoms related to hyperviscosity of the blood

Waldenstrom macroglobulinemia

Myeloproliferative disorder with the following: Obliterating marrow fibrosis Extensive deposition of collagen in marrow by non-neoplasticism fibroblasts (chief pathologic feature) Inappropriately released PDGF and TGF-beta (cause fibrosis and scarring)

Primary myelofibrosis

Lymphoid neoplasms are monoclonal meaning

All daughter cells are derived form the malignant progenitor share the same antigen receptor gene configuration and sequence Monoclonal - all daughter cells express the same configuration and sequence, implies malignancy

Most common cause of agranulocytosis/neutropenia | This causes patients to be more susceptible to ______

Drug toxicity | Bacterial infection

Prognosis of Hodgkin lymphoma

Curable in most cases

Which lymphoma spreads widely and less predictively

Non-Hodgkin’s

Most common trigger for hemophagocytic lymphohistiocytosis (HLH)

Infection - especially EBV

Reactive condition with cytopenias and signs/symptoms of systemic inflammation due to macrophage activation

Hemophagocytic lymphohistiocytosis

Dilated ER that stains to look like a “sky blue puddle” = granulocyte lineage effect seen in _______

Myelodysplastic syndromes (MDS)

Cortical thymomas rich in thymocytes are more likely to be associated with ____

Autoimmune disease

Burkitt lymphoma subtype with GI involvement | 15-20% latently infected with EBV

Sporadic (nonendemic) | AKA American Burkitt

Lab findings in primary myelofibrosis

Normocyctic, normochromic anemia with leukoerythroblastosis

Is LAP high or low in reactive states (e.g. infection)?

High

Neoplasm that commonly causes timorous masses throughout the skeletal system causing a “moth eaten” appearance

Multiple Myeloma

Splenic rupture is most commonly precipitated by ______

Blunt trauma (or by physician palpating)

Lymphoma that can present with lymphomatoid polyposis and can be misdiagnosed as UC

Mantle Cell Lymphoma

2 most common chromosomal rearrangements in acute myeloid leukemia (AML)

t(8:21) and inv(16)

Leukemia/lymphoma with the following characteristics: t(15:17) May have early life-threatening bleeding May have DIC (often fatal)

Acute promyelocytic leukemia (AML subtype)

Prognosis of t-MDS (therapy-related myelodysplastic syndrome)

Terrible | Median survival 5 months

Most important prognostic factor of Hodgkin lymphoma

Tumor stage | NOT histology

Basophilic leukocytosis is seen in

CML

In hairy cell leukemia, ____ have excellent tumor response in patients who fail chemotherapy

BRAF inhibitors

Prognosis of polycythemia Vera

Death within months if untreated | Phlebotomy extends survival to 10+ years

Multiple Myeloma: Bence-jones proteinuria: Excessive production and aggregation of M proteins, especially ___ or ___ -> hyperviscosity

IgA or IgG3

STAT3 mutation is seen in ____

Large granular lymphocytic leukemia

BRAF is characteristic of Hairy cell leukemia | BRAF is positioned immediately downstream of ___ in the ____ signaling cascade

RAS; MAPK

Most common leukemia of childhood | Most common cancer of children

ALL

Pathognomonic morphology of CLL

Proliferation centers - larger lymphocytes gathered in loose aggregates that contain mitotically active cells

Leukemia/lymphoma with the following pathogenesis/immunophenotype: ALK rearrangement on chromosome 2p23 Presence of ALK is pathognomic CD30+, CD 8 is anaplastic

Anaplastic large cell lymphoma (ALK+)

``` Hodgkin lymphoma subtype: Least favorable prognosis Least common subtype Elderly HIV+ and EBV+ Non-industrialized countries ```

Lymphocyte-depleted subtype

Chapter 13 Flashcards

(99 cards)