3. Conceptual classification
• Lukemia – based on cell type
– Acute/ chronic
– Myeloid/ lymphoid
• Lymphoma – malignant tumor of lymphoreticular
tissues:
– Hodgkin’s lymphoma/ disease
– Non Hodgkin’s lymphoma
4. Categories of haematopoeitic &
lymphoid neoplasia
1. Myeloid neoplasms
– Of myeloid cell lineage
– RBC, platelets, granulocytes and monocytes
2. Lymphoid neoplams:
– Of lymphoid cell lineage
– Lukemias and lymphomas of B,T or NK origin
3. Histiocytic neoplasms
– Proliferation of histiocytes
– E.g. langerhans cell histicytosis
5. Etiology
• Heridity – identical twins, family history, genetic
disorders
• Infections – HTLV-I, EBV, HCV, HIV, etc.
• Environmental factors – radiation, carcionogen, etc.
• Association with disease of immunity
6. Pathogenesis
• Genetic damage to single clone of target cells
– Proliferation of transformed clone
• Chromosomal translocations
– Lukemias have translocation of Philadelphia (Ph)
chromosome in 70-90% cases with CML (long arm of
chromosome 22 to chromosome 9)
• Maturition defect
– In acute lukemia
– AML – maturition defect beyond the myeloblast/
promyelocytic level
– ALL – lymphoblast level
7. Pathogenesis (Cntd…)
• Myelosuppression
– Accumulation of leukemic cells suppression of normal
hematopoeitic stem cells
• Organ infiltration
– Cells proliferate primarily in the bone marrow infiltrate
other sites
• Cytokines
– Reed sternberg cells secrete cytokines reactive
inflammation (in HL/ HD)
10. Introduction
• Lymphoid cells constitute immune system of the
body
• Differentiation and maturation occurs in lymphoid
tissues
• Neoplasm of cells leukemia
• Neoplasm of tissues lymphoma
• ALL, CLL, NHL, HD
11. WHO classification (1999)
1. Hodgkin’s disease
2. Precursor (immature) B- cell malignancies
3. Peripheral (mature) B-cell malignancies
4. Precursor (immature) T-cell malignancies
5. Peripheral (mature) T-cell malignancies
2 & 4 – Blastic type; e.g. ALL
3 & 5 – CLL and other lymphomas
12. General comments
• Incidence –
– NHL (62%); HD (8%); plasma cell disorders (15%); CLL (9%,
most common lymphoid leukemia) & ALL (4%)
• Diagnosis –
– Lymph node biopsy
– Blood picture
– Bone marrow Biopsy
• Staging – Ann Arbor
• Ancillary studies – CT scan, PET scan & gallium scan
• Immune abnormalities
14. Introduction
• Primarily arises within LN
• Extranodal secondaries
• Peak incidence – between 15-35 and after 50s
• Male> female
• Proliferation of lymphocytes, histiocytes, eosinophils
• Diagonistic feature presence of (Dorothy) Reed
Sternberg cells
15. Reed Sternberg Cell
• Diagonistic of Hodgkin’s lymphoma/ disease
• Classic RS cell –
– Large, with bilobed nucleus (mirror image) / miltilobed sometimes.
– Each nuclear lobe contains eosinophilic granular halo (owl eye
appearance)
• Lacunar type –
– Smaller with pericellular space (lacuna) d/t cell shrinkage
– Usu. In Nodulo sclerotic HD
• Polyploid (popcorn) type –
– Larger size with popcorn like lobulated nucleus
• Pleomorphic –
– Pleomorphic and atypical nuclei.
16. Classification of HD
Rye classification (1966)
1. Lymphocyte predominance type
2. Nodular sclerosis type
3. Mixed cellularity type
4. Lymphocyte depletion type
WHO classification
– Nodular lymphocyte predominant HD
– Classic HD (includes all 4 types in rye classification)
17. Pathophysiology
• Proliferation of tumor with only a small proportion of
cells are malignant
• Mostly normal lymphocytes
• RS cells – multinucleated giant cell mutations of T-
lymphocytes
• Infiltration leads to LN necrosis and fibrosis
18. Clinical Features
• Painless swelling in one of lymph nodes (usu.
Cervical region) with a h/o URTI
• Persistent fever, night sweats, fatigue, weight loss
• Malaise, pruritis, extreme pain, nerve irritation
• Absence of pulse d/t rapid enlargement of the LN
• Neck vein engorgement
• Enlargement of reteroperitoneal nodes, spleen and
liver
24. Clinical features
• Enlarged tonsils and adenoids
• Painless rubbery enlargement of lymphatic tissue,
usu. Cervical or supraclavicular nodes
• In children – cervical nodes affected first with
dyspnoea and coughing
• An advancing disease
• s/s specific to involved structure
• Systemic c/c – fatigue, malaise, weight loss, fever,
etc
25. Investigations
• Blood count – anaemia
• Uric acid level – elevated/ normal
• Sr. calcium – elevated
• Imaging – bone & CXR, lymphangiography, liver
and spleen scans, CT abdomen f/s/o metastasis
• LN biopsy – cell type revealed
• Biopsy of tonsil, bone marrow, liver or spleen
malignant cells
26. Treatment
• Radiation – esp in early and localized lesions
• Total nodal irradiation
• Chemotherapy
• Bone marrow transplant
• Blood transfusion (supportive)