Lymphoid Leukemia Flashcards by Kyra Catedral

Q

Mature Lymphoid Neoplasms
•__________: blood/bone marrow
•__________: lymph nodes, spleen, extranodal sites

A

Leukemias

Lymphomas

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Q

Adult T-cell leukemia/lymphoma
Prolymphocytic leukemia
Anaplastic large cell lymphoma
Mantle cell Ivionoma
Sézary syndrome
Diffuse large B-cell lymphoma
Peripheral T-cell lymphoma
Plasma cell (multiple) myeloma
Burkitt leukemia/lymphoma

A

Aggressive Disease

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Q

Chronic Ivmohocvtic leukemia/small Ivmohocvtic Ivmohoma
Large granular lymphocytic leukemia
Hairy cell leukemia
Mycosis fungoides
Lymphoplasmacytic lymphoma (Waldentröm macroglobulinemia)
Marginal zone lymphoma
Follicular lymphoma solitary plasmacytoma
Smoldering multiple myeloma

A

Generally Indolent Disease

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Q

Signs and Symptoms
- Asymptomatic to fulminant illness
- Nonspecific but are reflective of the organ involved

A

Mature Lymphoid Neoplasms

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Q

Unexplained weight loss (>10%BW in 6mos)
• Unexplained persistent fever (>38C)
• Recurrent night sweats

A

B symptoms

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Q

^ inflammatory cytokines (TNFa, IL1, IL6, INF gamma)

A

B symptoms

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

CBC

A

Lymphocytosis (absolute increase)

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

Document clonality
Establish an immunophenotype

A

Flow cytometry

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

detect genetic changes

A

FISH (cytogenetic studies)

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

Gold standard for lymphoma diagnosis

A

BMA/BM biopsy

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

• Evaluate organ systems for tumor involvement
• Indirect measure of tumor burden
• Assess prognosis

A

Biochemical analysis

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

Biochemical analysis

A

> Serum creatinine, Uric acid, Reticulocyte count, haptoglobin, DAT, LDH, Beta 2 macroglobulin, ESR, Hepatitis B, HIV

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

• Gold-standard for lymphoma

A

Excisional biopsy

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Q

Mature Lymphoid Neoplasms
Diagnostic Procedures

Imaging

• Greater localization
• More sensitive

A

• CT scan
• PET with FDG

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Q

Lymph Node Biopsy

Two main techniques:

•	Entire lymph node is removed and examined.
•	Preserves tissue architecture, which is crucial for subclassification of lymphomas.

•	Less invasive but may miss focal disease.
•	Works best when combined with flow cytometry.

A

Excisional Biopsy (Gold Standard)
Fine Needle Aspiration (FNA)

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Q

• Detects enlarged lymph nodes, spleen, or extranodal involvement.
• Helps classify disease as limited-stage vs. advanced-stage.

A

Computed Tomography (CT) Scans

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Q

• Uses fluorodeoxyglucose (FDG) to detect metabolic activity of tumors.

• High FDG uptake seen in aggressive lymphomas (DLBCL, HL, BL).

• Low or variable uptake in indolent lymphomas (CLL, MZL).

A

Positron Emission Tomography (PET-CT)

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Q

Staging systems are used to determine how far the disease has spread, which helps in treatment planning. The most widely used systems are:

A

Ann Arbor Staging System
Lugano Classification

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Q

Staging

is now preferred for non-Hodgkin lymphoma (NHL) due to biological differences between NHL and Hodgkin lymphoma (HL).

A

Lugano Classification

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Q

Originally developed for HL, this system classifies the disease based on the extent of nodal and extranodal involvement.

A

Ann Arbor Staging System

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Q

The_____ is a modified version of the Ann Arbor system designed specifically for NHL. It simplifies staging into limited and advanced disease categories

A

Lugano Classification

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Q

Ann Arbor

1 node involved or single extranodal site (E)

A

Stage I

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Q

Ann Arbor

≥2 nodes/groups or lymphatic structures on same side of diaphragm or

involvement of limited contiguous extralymphatic organ or tissue (IIE)

A

Stage II

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Q

Ann Arbor

Nodes on both sides of diaphragm (III)

with involvement of spleen
(IIIS) or

limited contiguous extralymphatic organ or tissue involvement (IIIE) or

both (IIIES)*

A

Stage III

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Ann Arbor Diffuse or disseminated foci of involvement of ***≥1 extralymphatic organ or tissue*** with or without associated lymphatic involvement

Stage IV

Lugano 1 node or group of nodes; ***single extranodal lesion without nodal involvement***

Stage I

Lugano ≥2 nodes or nodal groups on same side of diaphragm; stage l or Il by nodal extent with limited contiguous extranodal involvement Il bulky defined as a single or nodal abdominal mass of ≥10 cm or a mediastinal mass ≥⅓ the internal transverse diameter of the thorax

Stage II

Lugano Nodes on both sides of diaphragm Nodes ***above the diaphragm with spleen involvement***

Stage III

Lugano Additional noncontiguous extralymphatic involvement

Stage IV

is the primary tool for assessing prognosis in aggressive NHL.

International Prognostic Index (IPI) for NHL

The scoring systems were designed for follicular lymphoma (FL), recognizing that FL has a different disease course compared to aggressive lymphomas.

Follicular Lymphoma International Prognostic Index (FLIPI) The FLIPI and FLIPI2

is characterized by the ***uncontrolled proliferation of immature lymphocytes (lymphoblasts),*** leading to bone marrow failure and infiltration of organs.

Acute Lymphoblastic Leukemia (ALL)

It is the most common childhood cancer and can also occur in adults.

Acute Lymphoblastic Leukemia (ALL)

is a malignant disorder of the lymphoid precursor cells (lymphoblasts) in the bone marrow, blood, and other lymphoid organs.

Acute Lymphoblastic Leukemia (ALL)

• Most common childhood leukemia (75% of childhood leukemias) • Represents 25% of all childhood cancers

Acute Lymphoblastic Leukemia (ALL)

Acute Lymphoblastic Leukemia (ALL) • Peak incidence:______ • More common in______ • Risk increases with_____

2–5 years old males than females age (worse prognosis in adults)

• Peak incidence: 2–5 years old • More common in males than females • Risk increases with age (worse prognosis in adults)

Acute Lymphoblastic Leukemia (ALL)

• More common (85% of childhood ALL cases)

B-cell Acute Lymphoblastic Leukemia (B-ALL)

Signs and Symptoms: • Fatigue (due to anemia) • Fever (due to infections from neutropenia) • Mucocutaneous bleeding (due to thrombocytopenia) • Lymphadenopathy (enlarged lymph nodes) • Splenomegaly, hepatomegaly • Bone pain (due to leukemic infiltration) • Decreased neutrophil count (regardless of total WBC count)

B-ALL

• More common in adolescent males • Often presents with a large mediastinal mass (thymic involvement) • Less severe degree of leukopenia

T-cell Acute Lymphoblastic Leukemia (T-ALL)

• 11 subtypes with recurrent cytogenetic abnormalities

B-ALL/B-LBL

• Genetic abnormalities in 50–70% of cases

• T-ALL/T-LBL

T-ALL / T-lymphoblastic lymphoma (T-LBL) Two subcategories:

• Early T-precursor lymphoblastic leukemia (ETP-ALL) • T-ALL, Not Otherwise Specified (NOS)

Prognostic Factors in ALL 1. Age: • → Better prognosis • → Worse prognosis

Children (1–10 years) Infants (<1 year) and adults (>40 years)

Prognostic Factors in ALL Initial WBC count: • → Favorable • → Unfavorable

<50,000 cells/μL ≥50,000 cells/μL

Prognostic Factors in ALL Cytogenetics: • → Excellent prognosis • → Poor prognosis

ETV6::RUNX1 fusion & High hyperdiploidy BCR::ABL1 (Philadelphia chromosome), KMT2A rearrangement

• Size: ______the size of a normal lymphocyte • Cytoplasm: ***Scant (very little), deeply basophilic (blue-staining)*** • indistinct nucleoli

Small Lymphoblasts (1–2.5×)

with prominent nucleoli an nuclear membrane irregularities

Larger lymphoblast (2-3x)

Acute Lymphoblastic Leukemia Prognosis Dependent on:

• Age at time of diagnosis • Lymphoblast load • Immunophenotype • Genetic abnormalities

Acute Lymphoblastic Leukemia Prognosis : best prognosis

Children

Acute Lymphoblastic Leukemia Prognosis - Worse outcomes: - PBS lymphoblast_____ (2)

>20-30 x10%/L • Hepatosplenomegaly • Lymphadenopathy

Immunophenotypic Characteristics of Acute Lymphoblastic Leukemia Both B-ALL and T-ALL

• CD34+, TdT+, HLA-DR+

Immunophenotypic Characteristics of Acute Lymphoblastic Leukemia - CD19, CD20, CD22, CD24, CD79a, CD10, cytoplasmic y, PAX-5

• B cell markers:

Immunophenotypic Characteristics of Acute Lymphoblastic Leukemia • CD2, CD3, CD4, CD5, CD7, CD8, TdT

T cell markers

Immunophenotypic Characteristics of Acute Lymphoblastic Leukemia • (+) myeloid markers

ETP-ALL

CD34, CD19, cytoplasmic CD22, TdT

Early (pro/pre-pre) B-ALL

CD34, CD19, CD10, cytoplasmic CD22, TdT

Intermediate (common) B-ALL

CD34 +/-, CD19, cytoplasmic CD22, cytoplasmic u, TdT (variable)

Pre-B-ALL

CD2, CD3, CD4, CD5, CD7, CD8, TdT

T-ALL

Acute Lymphoblastic Leukemia Genetic and Molecular Findings • Gain of function mutations • T-ALL: NOTCH1 + INK4/ARF mutations

NOTCH1 gene

Acute Lymphoblastic Leukemia Genetic and Molecular Findings - ***worse prognosis*** among all ALLs

B-ALL/LBL with t(9;22)

Acute Lymphoblastic Leukemia Genetic and Molecular Findings • Young infants, poor prognosis

B-ALL/LBL with KMT2A rearrangement

Acute Lymphoblastic Leukemia Genetic and Molecular Findings • Derived from a B cell progenitor • ***Excellent prognosis (cure rate 90%)***

ETV6::RUNX1 translocation