OLFU COLLEGE OF MEDICINE CLASS 2017 3rd year

Hematology Lymphoid Neoplasms Dr. Tuy

LYMPHOID NEOPLASMS Categories of Lymphoid Neoplasms

 Compose of a wide spectrum of different  Acute and chronic lymphoid leukemia
morphologic and clinical syndromes - T cell or B cell
 Can originate from cells that are at stage prior to T  Malignant lymphomas
and B lymphocyte differentiation, from a primitive - Non-Hodgkin’s lymphoma
stem cell or from cells at stages of maturation after - Hodgkin’s disease
stem cell differentiation  REAL/WHO classification of lymphoid neoplasms

General Features
 Range from the most indolent to the most
aggressive of malignancies
 Arise from cells of the immune system at different
stages differentiation
 May present as leukemia or lymphoma

Epidemiology

B-cell Differentiation and Lymphoid Neoplasms

LYMPHOMAS
 A group of diseases caused by malignant
lymphocytes that accumulate in lymph nodes and
cause the characteristic features of
T-Cell Differentiation and Lymphoid Neoplasms lymphadenopathy
- Occasionally, they may spill over into blood
(leukemic phase) or infiltrate organs outside the
lymphoid tissue
 Major subdivision of lymphomas is into
- Hodgkin lymphoma
- Non-Hodgkin’s lymphoma
 based on the histological presence of Reed-
Sternberg (RS) cells in Hodgkin’s lymphoma

HODGKIN LYMPHOMA
 Probably of B cell origin

Epidemiology
 Bimodal distribution of age at diagnosis, one peak
incidence in 2nd decade and the other at 8th
decade

Trans by: URACIL MD 1

 OLFU COLLEGE OF MEDICINE CLASS 2017 3rd year

Hematology Lymphoid Neoplasms Dr. Tuy

Risk Factors Diagnosis

 HIV  Histologic examination of excised lymph node.
 Epstein-Barr virus  CD markers CD30 CD15 positive; negative for B-cell
antigen expression
Pathogenesis  Staging evaluation:
 Reed-Sternberg (RS) cells and associated abnormal - Chest radiograph
mononuclear cells are neoplastic - CT scan of the chest, abdomen, and pelvis
 Infiltrating inflammatory cells are reactive - Bone marrow biopsy

Clinical Features
 Can present at any age; rare in children; peak
incidence in young adults
 2:1 male predominance
 Most patients present with painless, non-tender,
asymmetrical, firm, discrete, and rubbery
superficial lymphadenopathy Diaphragmatic representation of the different cells in Hodgkin
- Cervical nodes – 60-70% Lymphoma
- Axillary nodes – 10-15%
- Inguinal nodes – 6-12% WHO Classification Hodgkin Disease
 Typically the disease is localized to a single  Classical Hodgkin’s disease
peripheral lymph node and progress by contiguity - Nodular sclerosis
within the lymphatic system - Mixed cellularity
 Splenomegaly and hepatomegaly may occur during - Lymphocyte depleted
the course of the disease - Lymphocyte rich
 Mediastinal mass ~ 10 % esp in nodular sclerosing  Nodular lymphocyte-predominant
type particularly in women
 Cutaneous manifestation - ~ 10% Histologic Types of Hodgkin Lymphoma
 Constitution symptoms in widespread disease
- B symptoms: fever, night sweat, and weight loss
- Pruritus – 25%
- Alcohol-induced pain
- Others- weight loss, profuse sweating at night

Hematological and Biochemical Findings

 Normocytic normochromic anemia
 neutrophilia in 1/3 of patients; eosinophilia
frequent
 lymphopenia and loss of cell-mediated immunity in
advanced disease
a. Classic RS cells
 Platelet normal or increased early phase, and
b. Mixed Cellularity
reduced – late stages
c. Nodular Sclerosing
 ESR and C-reactive protein elevated
 Elevated serum LDH

Trans by: URACIL MD 2

 OLFU COLLEGE OF MEDICINE CLASS 2017 3rd year

Hematology Lymphoid Neoplasms Dr. Tuy

Clinical Staging NON-HODGKIN LYMPHOMA

 A large group of clonal lymphoid tumors, most
commonly of B-cell origin (But may involve T-cell
and NK cell)
 Variable clinical presentation and natural history
 Characterized by an irregular pattern of spread and
a significant proportion of patients develop
extranodal disease
 Represent the 5th most common malignancy in
developed countries

Epidemiology
 More frequent in the elderly
 More common in men

Clinical Staging – Ann Arbor Staging WHO Classification of Lymphoid Neoplasms – B Cell

Stage number is followed by letter A or B indicating absence or

presence of fever, night sweats and weight loss

Treatment
 Radiotherapy
 Combined modality therapy
 Combination chemotherapy
- doxorubicin, bleomycin, vinblastine, and
dacarbazine (ABVD)
- mechlorethamine, vincristine, procarbazine, and
prednisone (MOPP)

Prognosis

Trans by: URACIL MD 3

 OLFU COLLEGE OF MEDICINE CLASS 2017 3rd year

Hematology Lymphoid Neoplasms Dr. Tuy

Cell of Origin Investigations and Staging Procedures

 Initial studies
- History and physical examination
 Biopsy specimen
 Pathologic diagnosis
 Flow cytometry
- Immunohistochemistry
- Cytogenetic analysis
- CT/PET scans
 Neck
 Chest
 Abdomen
 Pelvis

Characteristic Immunophenotype and Cytogenetics of

B-Cell Lymphomas
Clinical Behavior
 Low (indolent) and high-grade NHL
 Leukemias and lymphomas

Etiology / Risk Factor

 Immunosuppression
- patients with HIV infection
- organ transplant recipient
- inherited immune deficiencies
 Autoimmune diseases
- Sicca syndrome
- Rheumatoid arthritis
 Infectious agents
 Environmental factors

Infectious Agents Associated with NHL

Staging Procedures
 Additional studies
- Immunoglobulin and T cell receptor gene
rearrangement studies
- Polymerase chain reaction for Bcl-1 and Bcl-2
- Ultrasonography and MRI to clarify
abnormalities
- CT scan or MRI of brain if neurologic signs or
symptoms
- Analysis of cerebrospinal fluid if neurologic signs
Clinical Features or symptoms
 Superficial lymphadenopathy - Gastrointestinal studies if Waldeyer ring
 Constitutional symptoms involvement
 Oropharyngeal involvement
 Symptoms due to bone marrow involvement ◦○ END ○◦
 Abdominal diseases Sources:
 Other organs - Dr. Tuy 2015 Lecture

Trans by: URACIL MD 4