Diffuse large B cell Lymphoma|What is diffuse large B cell lymphoma?Review- usmle,neetpg, plab,fmge
Diffuse large B cell Lymphoma|What is diffuse large B cell lymphoma?Is diffuse large B cell lymphoma curable?
This video enumerates the salient features of Diffuse large B cell lymphoma with an interesting case study. High yield points are stressed.
Watch now for a high-yield review.
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A high grade, aggressive but potentially curable B-cell lymphoma.
Clinical pathology:
Most occur in older patients, but has a wide age range, including children.
Patients usually present with a rapidly enlarging mass at either a single**** nodal or extranodal site.
Symptoms if present are due to mass effect.
DLBCL is very aggressive, but usually responds to chemotherapy, with lasting remissions seen in ~50% of patients.
Progresses rapidly if untreated.
Morphology:
Gross: Large, irregular infiltrating lesion in nodal/extranodal location.
Microscopy:
Composed of large cells with a diffuse growth pattern***.
Tumor cells express B cell markers:
CD 10, CD 19 and CD20***
Variable expression of Bcl6, Bcl2, Myc
Etiology:
Some DLBCL show t(14;18)*** indicating transformed follicular lymphoma
BCL6-oncogene (3q27)- rearrangement is the most frequent chromosomal abnormality in diffuse large B-cell lymphoma (DLBCL)
Some are transformed CLL/SLL(Richeter’s Syndrome)
Some are associated with immunodeficiency states****
Immunodeficiency associated large B cell lymphoma***:
Occur in patients with HIV infection or transplant recipients
Tumor cell infected with EBV.
Body cavity based large B cell lymphoma**:
Occur in patients with HIV infection
Presents as pleural or ascitic effusion
Tumor infected with human herpes virus (HHV-8).
Treatment and Prognosis:
Aggressive combination chemotherapy helps in achieving complete remission and cure in 40-50% patients.
The extent of disease spread at the time of diagnosis, largely determines the prognosis.
Myc translocation imparts a bad prognosis
CAR T cells directed against the B-cell antigen CD19 are used to treat patients with relapsed refractory DLBCL.
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