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Essential Hematology > Chapter 17: Non-Hodgkin's lymphoma > Flashcards

Chapter 17: Non-Hodgkin's lymphoma Flashcards

1
Q

What is the primary sign of Non-hodgkin’s lymphoma?

A

superficial, asymmetric, painless lymphadenopathy.

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2
Q

What are the constitutional symptoms of NHL?

A

(1) Fever
(2) night sweats
(3) weight loss
They are commonly associated with disseminated disease.

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3
Q

What are often the presenting features of NHL?

A

(1) Anemia
(2) Neutropenia (leading to infections)
(3) Thrombocytopenia (leads to bleeding/purpura)

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4
Q

What are some gross pathological signs of NHL?

A

(1) lymphadenopathy
(2) Hepatosplenomegaly
(3) Skin involvement (mycoses fungoides, and Sezary syndrome)

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5
Q

Generally speaking how are NHLs identified?

A

Lymph node biopsy with morphological, immunophenotypic, and genetic investigation.

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6
Q

How does light chain clonality impact the diagnosis of NHL?

A

Polyclonal light chains are indicative of chronic inflammation. Monoclonal light chains are indicative of malignancy.

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7
Q

What are the hematological findings associated with NHL?

A

(1) Normochromic, Normocytic anemia
(2) Autoimmune hemolytic anemia
(3) Neutropenia (advanced disease)
(4) Thrombocytopenia

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8
Q

What are the biochemical findings associated with NHL?

A

(1) increased LDH
(2) Increased uric acid.
(3) paraprotein on Ig electrophoresis.

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9
Q

What translocation is associated with Burkitt’s lymphoma?

A

t(8; 14) Burkitt’s lymphoma

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10
Q

What translocation is associated with follicular lymphoma?

A

t(14; 18)

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11
Q

What translocation is associated with Mantle cell lymphoma?

A

t(11; 14)

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12
Q

What translocation is associated with anaplastic large cell lymphoma?

A

t(2; 5)

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13
Q

What methods are used to stage NHLs?

A

(1) imaging (CT, XR, MRI, PET)

(2) Biopsy, BM aspiration, Trephine biopsy.

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14
Q

What are the low grade NHLs?

A

(1) Follicular lymphoma
(2) Lymphocytic lymphomas
(3) Lymphoplasmacytoid lymphomas
(4) Mantle cell lymphoma
(5) Marginal zone lymphoma

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15
Q

What is the most common form of NHL?

A

Follicular lymphoma

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16
Q

What is the pathogenesis of follicular lymphoma?

A

B cells undergo malignant transformation by the t(14; 18) translocation, which causes the constitutive activation of the Bcl-2 gene.

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17
Q

What is the general age range for follicular lymphoma?

A

Middle aged to elderly

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18
Q

What is the median survival from diagnosis for follicular lymphoma?

A

10 years

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19
Q

What is the presentation of follicular lymphoma?

A

Painless lymphadenopathy with most patients presenting in stage III or IV.

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20
Q

What is the most effective chemotherapy against follicular lymphoma?

A

CVP (chlorambucil/cyclophosphamide, vincristine, and prednisolone.) achieves a response in up to 90% of patients.

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21
Q

If chemotherapy is not effective enough what alternative therapies can be considered for follicular lymphoma?

A

(1) Rituximab
(2) Autologous stem cell transplant
(3) Allogenic stem cell transplant ( in younger patients)

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22
Q

What are lymphocytic lymphomas?

A

Lymphocytic lymphomas are considered a tissue phase of CLL and are treated as such.

23
Q

What are lymphoplasmacytoid lymphomas?

A

Lymphomas associated with the production of monoclonal immunoglobulin M. Also called Waldenstroms macroglobulinemia.

24
Q

What are the complications associated with Lymphoplasmacytoid lymphomas?

A

Anemia and hyperviscosity.

25
Q

How is Lymphoplasmacytoid lymphoma treated?

A

Oral chlorambucil, fludarabine, or monoclonal antibodies. Plasma exchange can reduce viscosity.

26
Q

What is mantle cell lymphoma?

A

A malignancy derived from pre-germinal B cells located in primary follicles or in the mantles of secondary follicles.

27
Q

What is the characteristic phenotype of mantle cell lymphoma?

A

(1) CD19+
(2) CD5+
(3) CD22+
(4) CD23-

28
Q

What is the pathogenesis of mantle cell lymphoma?

A

t(11; 14) translocation that merges Bcl-1 with an Ig heavy chain gene promoter.

29
Q

What is the clinical presentation of Mantle cell lymphoma?

A

(1) lymphadenopathy
(2) bone marrow infiltration
(3) tumor cells in the peripheral blood.

30
Q

What histological findings are associated with mantle cell lymphoma?

A

Cells with characteristically angular nuclei.

31
Q

What is the prognosis for mantle cell lymphoma?

A

Even though it is considered low grade the prognosis for mantle cell lymphoma is poor. The median survival is 3 years post diagnosis.

32
Q

How is mantle cell lymphoma treated?

A

CVP or CHOP or fludarabine with rituximab.

33
Q

What are marginal zone lymphomas?

A

Lymphomas that are typically extranodal and localized.

34
Q

What is the pathogenesis of marginal zone lymphomas?

A

chronic inflammation of the tissue involved.

35
Q

What is the most common mantle cell lymphoma?

A

Gastric MALT lymphoma caused by H. pylori infection.

36
Q

How are marginal zone lymphomas treated?

A

Most can be treated with chemotherapy and rituximab. Splenectomy can be used for splenic marginal zone lymphoma.

37
Q

What are the high grade NHLs?

A

(1) Diffuse large B-cell lymphoma
(2) Burkitt’s lymphoma
(3) Lymphoblastic lymphomas.

38
Q

What are diffuse large b cell lymphomas?

A

Malignant disorders characterized by rapidly progressive lymphadenopathy due to fast cellular proliferation.

39
Q

What features of diffuse b cell NHL are associated with a poor prognosis?

A

(1) High age
(2) More extranodal sites
(3) higher serum LDH
(4) Bulky disease
(5) AIDS

40
Q

What features of diffuse B cell NHL are associated with a good prognosis?

A

(1) germinal center origin

2) t(3; 27

41
Q

How is diffuse large B cell NHL treated?

A

R-CHOP is the first line therapy. Prophylactic therapy for CNS disease with MTX may also be considered.

42
Q

What is the prognosis for diffuse large b cell lymphoma?

A

Long term survival is approximately 65%.

43
Q

What is Burkitt’s lymphoma?

A

The lymphomatous correlate of L3 ALL.

44
Q

Where is Burkitt’s lymphoma endemic?

A

Africa and the caribbean where there is chronic malaria exposure. It is associated with EBV infection.

45
Q

What is the pathogenesis of Burkitt’s lymphoma?

A

t(8; 14) moves the C-MYC oncogene under the control of an Ig heavy chain promoter thus over expressing the C-MYC oncogene.

46
Q

What chemotherapy regimen is highly effective against Burkitt’s lymphoma?

A

High dose methotrexate and cyclophosphamide.

47
Q

What is peripheral t cell NHL?

A

Malignancy derived from post thymic T cells. Treated with CHOP. The prognosis is poor.

48
Q

What is angioimmunoblastic lymphadenopathy?

A

It is a T cell malignancy of the elderly associated with

(1) lymphadenopathy
(2) Hepatosplenomegaly
(3) Skin rashes
(4) Polyclonal IgG increase

49
Q

What is mycosis fungoides?

A

Mycosis funcoides is a chronic cutaneous T cell lymphoma that presents with

(1) Severe pruritis
(2) psoriasis like lesions

50
Q

What is Sezary syndrome?

A

A T cell lymphoma with circulating T-lymphoma cells that presents with

(1) dermatitis
(2) generalized lymphadenopathy
(3) CD4+ cells with folded nuclear chromatin.

51
Q

What is Adult T cell lymphoma?

A

A malignancy of T cells that is caused by the HTLV-1. It presents with

(1) hepatosplenomegaly
(2) cutaneous infiltrations
(3) hypercalcemia

52
Q

What are angiocentric lymphomas?

A

Typically involve the sinuses or gluten induced enteropathy

53
Q

What is anaplastic large cell lymphoma?

A

A T cell malignancy characterized by CD30+ cells and the t(2; 5)

Essential Hematology (22 decks)

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