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48 Cards in this Set

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  • Back
Define Lymphoblastic Lymphoma
Neoplasm of precursor T cells
Neoplasm of precursor T cells that affects Adolescent Males and presents with an Anterior Mediastinal mass
Lymphoblastic Lymphoma
How does Lymphoblastic Lymphoma present usually?
Anterior Mediastinal Mass in Adolescent Males
- can present with pulmonary symptoms related to the mass
-compress the trachea and major bronchi and lead to airway obstruction
Mimics B cell ALL morphologically
Lymphoblastic Lymphoma
Define Burkitt Lymphoma
highly aggressive neoplasm of MATURE B cells
Describe the pathogenesis of Burkitt Lymphoma
1. Viral infection (HIV or EBV)
2. C-myc proto-oncogene overexpression
3. t(8;14) translocation
Describe the pathology of Burkitt Lymphoma
1. Diffuse infiltrate of Intermediate size Lymphocytes = effaces the lymph node architecture
2. High mitotis activity
3. Tumor cell apoptosis
4. Starry sky pattern
Describe the Endemic type of Burkitt Lymphoma
1. Africa or New Guinea
2. Children are affected
3. Mandible involvement
4. associated with EBV
Describe the Sporadic type of Burkitt Lymphoma
1. USA
2. Children or young adults
3. Abdominal mass
4. associated with HIV
Neoplasm of CD4+ cells that is endemic to Japan and Caribbean
Adult T cell Leukemia/Lymphoma
What does the pathogenesis of Adult T cell Leukemia/Lymphoma involve?
HTLV-1 infection
List the clinical symptoms of Adult T cell Leukemia/Lymphoma (3)
1. Skin lesions
2. HYPERCALCEMIA
3. Enlargement of the Spleen, Lymph Nodes, and Liver
Adult T cell Leukemia/Lymphoma
Hyperlobated "4-leaf clover" lymphocytes in the peripheral blood
What is the prognosis of Adult T cell Leukemia/Lymphoma?
median survival < 1 year
Define Mycosis Fungoides/Sezary Syndrome
Skin neoplasm of CD4+ T cells
Skin neoplasm of CD4+ T cells that produces skin plaques, then nodules, then tumors
Mycosis Fungoides
Skin neoplasm of CD4+ T cells that presents with Diffuse Erythroderma (redness)
Sezary Syndrome
What is the prognosis of Mycosis Fungoides / Sezary Syndrome?
median survival > 5 years
What is the treatment for Mycosis Fungoides / Sezary Syndrome?
Chemotherapy

Light Therapy = kills lymphocytes --> palliates disease
Describe the histology of Mycosis Fungoides / Sezary Syndrome
Epidermotropic lymphocytes
- Pautrier microabscesses of the skin
- Cerebriform nucleus (nuclear membrane folding) of lymphocytes
List the 3 general features of Hodgkin Disease
1. B cell neoplasm

2. Neoplastic cell is Reed-Sternberg Cell

3. CONTIGUOUS spread = goes from one node to adjacent lymph node
Describe Nodular Sclerosis Hodgkins Disease (5)
1. Most common HD = 70%
2. affects YOUNG FEMALES
3. Anterior Mediastinal mass
4. Lymphoid nodules surrounded by fibrotic bands
5. Lacunar Cells (RS variant)
Describe Mixed Cellularity HD
1. affects Males
2. comprises 25% of HD's
3. Diffuse Polymorphous infiltrate = lymphocytes, eosinophils, plasma cells, histiocytes
Describe Lymphocyte Predominant HD
1. Least common = 5%
2. Males most affected
3. Nodules of small mature Lymphocytes = Popcorn cells (RS variant)
4. associated with "good prognosis"
List the clinical features of Hodgkin Disease
1. Localized lymphadenopathy (especially Axillary & Cervical)
2. B symptoms
-fever, night sweats, weight loss
3. Pruritis
4. Alcohol induced lymph node pain
Describe Stage I HD
Single Lymph Node group
Describe Stage II HD
2 or more lymph node groups on the same side of the Diaphragm
Describe Stage III HD
Lymph Node regions on both sides of Diaphragm
Describe Stage IV HD
Disseminated disease
Describe the treatment for the Stages of HD
Stage 1 & 2 can have Radiation alone

Stage 3 & 4 need radiation and Chemo combo
What is the survival rate for Stage I and II HD?
90% 5 year survival
What is the survival rate for Stage IV HD?
60-70% 5 year survival
What are the complications of HD?
Secondary Malignancy
1. Carcinoma = lung, breast, stomach
2. Hematologic = AML, MDS
3. Sarcoma, Melanoma

**due to radiation and chemotherapy
Burkitt Lymphoma
-Starry-sky appearance
-large cells with clear cytoplasm = macrophages engulfing apoptotic cells
What disease?
Mycosis Fungoides/Sezary Syndrome

Pautrier Microabscess
What disease?
What is this called?
Reed-Sternberg Cell = owl-eye binucleated cell

Hodgkin Disease
What is the arrow pointing at?

What disease?
What is the survival rate for Stage I and II HD?
90% 5 year survival
What is the survival rate for Stage IV HD?
60-70% 5 year survival
What are the complications of HD?
Secondary Malignancy
1. Carcinoma = lung, breast, stomach
2. Hematologic = AML, MDS
3. Sarcoma, Melanoma

**due to radiation and chemotherapy
-Nodular Sclerosis HD
-Lymphoid nodules surrounded by fibrotic bands
-Females
What specific disease?
How do you know?
What gender?
Reed-Sternberg cell

Hodgkin Disease
What is this showing?
What disease?
Lacunar cells

Nodular Sclerosis
What are these cells called?

What disease?
Popcorn Cell = large mononuclear cells with high irregularly folding nucleus

Lymphocyte predominance HD
What are these cells called?

What disease?
Mycosis Fungoides / Sezary Syndrome
-Cerebriform nucleus = nuclear membrane folding

**The cells with the nuclear cleft are malignant CD4 T helper cells, or Sezary cells. They stain positive for PAS (not demonstrated in this slide) and have PAS positive chunks of material located around the nucleus ("ring around the collar" analogous to ringed sideroblasts in sideroblastic anemias").
What disease are these cells found in?
B. Hodgkin Disease
-
D. Spleen

**Hairy Cell Leukemia results in massive Splenomegaly

Mandible = Endemic Burkitts
Skin = ATLL or Mycosis Fungoides/Sezary Syndrome
Stomach = MALToma or Burkitt
-
A 22-year-old man presents with a painless lump in his neck. Upon further questioning he has had a low-grade fever, drenching night sweats, 14 lb. weight loss over the past 6 wks, and his skin is itchy. He also states that after a night of drinking he has pain in the lump in his neck
Hodgkin Lymphoma
-PAINLESS LYMPHADENOPATHY
-Pruritis
-Splenomegaly
-Low grade fever, night sweats, weight loss
T cell cancer associated with Hypercalcemia
Adult T cell Leukemia/Lymphoma