30. High grade B-cell lymphomas (BL, DLBCL). Plasma cell neoplasms

What are the aggressive/high grade B-cell lymphomas?

- Burkitt lymphoma

- Diffuse large B-cell lymphoma (DLBCL)

Burkitt lymphoma translocation?

All forms of Burkitt lymphoma are associated with translocation of the C-MYC gene

- t(8;14) - IgH/MYC

- t(2;8) - IgK/MYC

- t(8;22) IgL/MYC

What will the translocation of MYC lead to?

Increased expression of MYC

- group of proto-oncogenes, which codes of transcription factors

What are the subgroups of Burkitt lymphoma?

- Endemic

- Sporadic

- Immunecompromised-related

Endemic Burkitt's Lymphoma?

Associated with EBV-infection

- children and young adults in equatorial Africa and South-America

- head and neck area

Sporadic Burkitt's lymphoma?

Associated with EBV-infection in 30% (rest is sporadic)

- children and young adults in western countries

- abdominal mass

Immunecompromised-related Burkitt's lymphoma?

Associated with HIV/AIDS, and other immune-compromised states

Diffuse large B-cell lymphoma translocation?

No mutation or translocation that is always implicated

- one or more of the genes MYC, BCL-2, BCL-6 are commonly mutated or translocated

Diffuse large B-cell lymphoma can be?

- Primary (de novo)

- Secondary

Secondary Diffuse large B-cell lymphoma?

Richter transformation;

- CLL

- FL

- MZL

DLBCL features?

Often forms rapidly growing tumors

- approx. 60% of them are extranodal

Extranodal DLBCL are usually found where?

- CNS

- GIT

- Mediastinum

- Skin

What are plasma cell neoplasms?

Neoplasms originating from plasma-cells

- belong to the "other type" group of mature, non-Hodgkin B-cell lymphomas

Diagnosis of plasma cell neoplasms?

- Based on the presence of monoclonal immunoglobulins in the serum = M-proteins

- Based on the detection of Bence-Jones proteins in the urine

Why will there be monoclonal immunoglobulins in the serum in plasma cell neoplasms?

Because the plasma cells produce immunoglobulins

- since the neoplasm originates from one plasma cell, all the tumor cells produce the same immunoglobulin

What are Bence Jones proteins?

Parts of the immunoglobulins (often Ig light-chain proteins)

- that are detected in the urine

What can plasma cell neoplasms also be called? why?

Monoclonal gammopathies

- because of the large amounts of immunoglobulins in the serum

Which disease can occur in plasma cell neoplasms? why?

AL amyloidosis

- because of the high level of immunoglobulin light chains in the blood

What are the different types of plasma cell neoplasms?

- Multiple myeloma

- Solitary plasmacytoma

- Monoclonal gammopathy of unknown significance

- Lymphoplasmocytic lymphoma

What is multiple myeloma?

Malignant proliferation of plasma cells into the bone marrow

What are the symptoms of multiple myeloma?

CRAB

- Hypercalcaemia

- Renal failure

- Anaemia

- Bone lesions

Why will there be hypercalcaemia in multiple myeloma?

Because of the osteolysis

Why will there be renal failure in multiple myeloma?

The kidneys will be damaged by the M-proteins and Bence-Jones proteins

Why will there be anaemia in multiple myeloma?

Because of the infiltration of the bone marrow

What is a solitary plasmacytoma?

An early stage of multiple myeloma, where there are yet no systemic symptoms

- occurs as a solitary mass of clonal plasma cells

What is monoclonal gammopathy of uknown significance?

An asymptomatic monoclonal gammopathy

- there are M-proteins in the blood, but no organ damage, lymphoma or leukaemia