Lymphoma-All you Need to know

in ONE hour

Cathleen CookPediatric Hematology/ OncologyMarch 12, 2014

Ranking of pediatric malignancies for children aged 0-14 years

http://www.meb.uni-bonn.de/cancer.gov/CDR0000062872.html

Leukemia

Brain tumors

Lymphoma

Ranking of pediatric malignancies for children aged 15-19 years

http://www.meb.uni-bonn.de/cancer.gov/CDR0000062872.html

Carcinomas and Melanoma

Brain tumors

Lymphoma

Hodgkin Lymphoma

Non-Hodgkin Lymphoma

Late Effects

Juan PabloNicole

Objectives

Epidemiology of Hodgkin Lymphoma

O 5% of cancers ≤14 years of ageO 15% of cancers ≥14 years of age

O Bimodal age distribution

Associations with Hodgkin Lymphoma

EBVCMV

HHV6

What is Hodgkin Lymphoma?

O Cancer of the lymphoreticular system

Hodgkin Lymphoma Explained

http://www.youtube.com/watch?v=saYsSIsBObw&list=PLlacjr_GktwC9JDvcxvgGKQ4jMq2JAE0L

Reed-Sternberg (RS) Cells = Hallmark of classical Hodgkin Lymphoma

Other Subtypes of Hodgkin Lymphoma

To where can Hodgkin Lymphoma metastasize?

SpleenLiver

Bone MarrowBone

Brain

Symptoms of Hodgkin Lymphoma

O Painless, nontender, firm, rubbery lymph node

O CoughO Weight lossO FeverO Night sweatsO Pruritus (itching)O Anorexia (appetite loss)O Tiredness/ LethargyO Pain WORSENING with Alcohol

B symptoms

1:30 sec. --- 5:00

Nicole’s Symptoms

http://www.youtube.com/watch?v=vsSrdEYjcpc

So how do we diagnose Nicole?

Physical Exam

So how do we diagnose Nicole?

Sites of Disease

Source: Metzger M. Hodgkin Lymphoma Lecture. 2009

So how do we diagnose Nicole?

LabsCBC with differential

CMP

LDH

Uric Acid

ESR

CRP

Ferritin

EBV titers

CMV titers

Diagnostic Work Up: Chest X-ray

Diagnostic Work Up: CT CAP

Diagnostic Work Up: PET Scan

Diagnostic Work Up: Bilateral Bone Marrow Biopsy/Aspirate

http://www.dana-farber.org/Adult-Care/Treatment-and-Support/Hodgkin-Lymphoma.aspx#Cancer_Summary

Diagnostic Work Up: Excisional Lymph Node Biopsy

http://www.flickr.com/photos/santoshpath/5453069538/http://tessascancerjourney2.blogspot.com/2010/11/lymph-node-biopsy.html

Summary of Nicole’s diagnostic work up for Hodgkin Lymphoma?• Physical Exam• CBC• CMP• LDH• Uric Acid• ESR• CRP• Ferritin• EBV titers• CMV titers

• Chest Xray- PA/L• CT scan (CAP)• PET scan• Bone marrow aspirate/biopsy• Excisional LN biopsy

So how do we stage Hodgkin Lymphoma?

A= no “B symptomsB= fever (>38C), unexplained weight loss (>10%), drenching night sweatsE= extranodal extensionS= splenic involvement Source: Metzger M. Hodgkin Lymphoma Lecture. 2009

So how do we treat Hodgkin Lymphoma?

CHEMOTHERAPYO Prednisone (P)O Vinblastine (V)O Bleomycin (B)O Doxorubicin (A)O Vincristine (O)O Procarbizine (P)O Etoposide (E)O Cyclophosphamide

(C)

COPPCOPDAC OPPAOEPA

ABVDVAMP

So how do we treat Hodgkin Lymphoma?

RADIATION

Source: Metzger M. Hodgkin Lymphoma Lecture. 2009

Hodgkin Lymphoma

Non-Hodgkin Lymphoma

Late Effects

Juan PabloNicole

Objectives

Epidemiology of Non-Hodgkin Lymphoma (NHL)

O 60% of all lymphomas in children and adolescents

O 8-10% of cancers between 5-19 yo

O >70% have advance disease at diagnosis

Source: Kliegman R, Staton B, et al. Nelson Textbook of Pediatrics Ebook. 19 th edition. 2011. pg 8002.

Associations with Non-Hodgkin Lymphoma

EBVHIV BLOOM SYNDROME

ATAXIA-TELANGIECTASIA

WISKOTT-ALDRICH SYNDROMESCIDDe

NOVO

Source: Kliegman R, Staton B, et al. Nelson Textbook of Pediatrics Ebook. 19 th edition. 2011. pg 8002.

What is Non-Hodgkin Lymphoma?

O Cancer of the lymphocytes

Non-Hodgkin Lymphoma Explained

https://www.youtube.com/watch?v=x3c9dy3MPZo

Subtypes of Non-Hodgkin Lymphoma

38% Burkitt lymphoma (BL)

29% Lymphoblastic lymphoma (LL)

20% Diffuse large B-cell lymphoma (DLBCL)

10% Anaplastic large cell lymphoma (ALCL)

0-14 years

Source: Kliegman R, Staton B, et al. Nelson Textbook of Pediatrics Ebook. 19 th edition. 2011. pg 8003.

Subtypes of Non-Hodgkin Lymphoma

37% Diffuse large B-cell lymphoma (DLBCL)

21% Burkitt lymphoma (BL)

19% Lymphoblastic lymphoma (LL)

17% Anaplastic large cell lymphoma (ALCL)

15-19 years

Source: Kliegman R, Staton B, et al. Nelson Textbook of Pediatrics Ebook. 19 th edition. 2011. pg 8003.

Clinical Presentation of Non-Hodgkin Lymphoma

MASSES

Jaw swelling

Abdominal masses

Orbital swelling

Chest masses

Paraspinal masses

Clinical Presentation of Non-Hodgkin Lymphoma

http://www.youtube.com/watch?v=Zx9SjYTr7CU

Juan Pablo’s Symptoms

Initial 1:00min

Non-Hodgkin Lymphoma- Tumor Lysis Syndrome

Tumor masses (similar to leukocytosis with leukemia)

http://www.nejm.org.ezproxy.etsu.edu:2048/doi/pdf/10.1056/NEJMra0904569

Tumor Lysis Syndrome

http://www.nejm.org.ezproxy.etsu.edu:2048/doi/pdf/10.1056/NEJMra0904569

Uric AcidPhosphatePotassium

Calcium SodiumNO CHANG

E

Tumor Lysis Syndrome

http://www.nejm.org.ezproxy.etsu.edu:2048/doi/pdf/10.1056/NEJMra0904569http://www.nytimes.com/health/guides/disease/acute-gouty-arthritis/print.html

Uric AcidPhosphatePotassium RISK OF

Acute Kidney Injury-stones

-renal failure

Tumor Lysis Syndrome

http://www.nejm.org.ezproxy.etsu.edu:2048/doi/pdf/10.1056/NEJMra0904569http://www.pinterest.com/pin/90986854945562677/http://www.aaemrsa.org/communication/modernresident/2012/images/hands-lg.jpg

Calcium RISK OF

Chvostek sign Trousseau's sign

Emergency Presentations of Non-Hodgkin Lymphoma

So how do we diagnose Juan Pablo?

Physical Exam

So how do we diagnose Juan Pablo?

LabsCBC with differential

CMP

LDH

Uric Acid

ESR

CRP

Ferritin

EBV titers

CMV titers

Diagnostic Work Up: Chest X-ray

Diagnostic Work Up: CT CAP

http://tmcr.usuhs.edu/tmcr/chapter8/differential3.htmhttp://www.radiologyschools.com/Radiology-Courses/gu/kidneys/lymphoma.html

Diagnostic Work Up: PET Scan

http://www.hindawi.com/journals/crim/2012/239719/fig2/https://www.mi-lifenet.com/ImagingCenter/zportal/portals/phys/clinical/petct_case_studies/lymphoma/lymphoma_case3

Diagnostic Work Up: Bilateral Bone Marrow Biopsy/Aspirate

http://www.dana-farber.org/Adult-Care/Treatment-and-Support/Hodgkin-Lymphoma.aspx#Cancer_Summary

Diagnostic Work Up: Least Invasive Biopsy

http://www.umphysicians.org/cancercare/cancer-information/cancer-library/CDR269299.html#_1

Diagnostic Work Up: Lumbar Puncture

http://www.webmd.com/cancer/tc/brain-cancer-primary-central-nervous-system-lymphoma-treatment-patient-information-nci-pdq-general

Summary of diagnostic work up for Non-Hodgkin Lymphoma?• Physical Exam• CBC• CMP• LDH• Uric Acid• ESR• CRP• Ferritin• EBV titers• CMV titers

• Chest Xray- PA/L• CT scan (CAP)• PET scan• Lumbar puncture• Bone marrow aspirate/biopsy• Least invasive Biopsy

So how do we treat Non-Hodgkin Lymphoma?

CHEMOTHERAPYWITH INTRA-THECAL

CHEMOTHERAPY

O Cyclophosphamide (C)O Vincristine (O)O Prednisone (P)O Doxorubicin (A)

COPAD O Methotrexate (M)O Hydrocortisone (H)

O Cytarabine (A)

+Radiation

Hodgkin Lymphoma

Non-Hodgkin Lymphoma

Prognosis andLate Effects

Juan PabloNicole

Objectives

What are prognoses for Lymphoma?

Hodgkin Lymphoma

> 85% - 93+ %

NON-HODGKIN LYMPHOMA

90-95% localized60-90% advanced

What are Late Effects for Lymphoma?

Based on…

Late Effects of Chemotherapy- Steroids

Reduced bone mineral density (BMD)

Avascular necrosis (AVN)

Growth asymmetry/Decreased bone growth

Cataracts

Dental caries

Late Effects of Chemotherapy-

Vincristine/Vinblastine

Peripheral sensory or motor neuropathy Areflexia Weakness Foot drop Paresthesia

Dental caries

Late Effects of Chemotherapy-

Bleomycin

Interstitial pneumonitisPulmonary fibrosis

Dental caries

Late Effects of Chemotherapy-

Doxorubicin

CardiomyopathyArrhythmiasSubclinical left ventricular dysfunction (by ECHO or MUGA)

DOSE correlates with cardiotoxicity

Secondary leukemia (AML/MDS)

Dental caries

Late Effects of Chemotherapy-

Etoposide

Secondary leukemia (AML/MDS)

Dental caries

Late Effects of Chemotherapy-

Cyclophosphamide

Hemorrhagic cystitisBladder fibrosis

Male GU dysfunction/infertility

Female GU dysfunction/infertility

Cardiomyopathy

Secondary leukemia (AML/MDS)Secondary malignancy (solid tumor)

Dental caries

Late Effects of Radiation to NECK

Hypothyroidism

Late Effects of Radiation to CHEST

Restrictive Lung Disease

Late onset breast cancer

Cardiac dysfunctionPericarditisPericardial fibrosis

Growth asymmetry

Secondary malignancy (solid tumor)

O Painless, nontender, firm, rubbery lymph nodeO CoughO Weight lossO FeverO Night sweatsO Pruritus (itching)O Anorexia (appetite loss)O Tiredness/ Lethargy

B symptoms

• Physical Exam• CBC• CMP• LDH• Uric Acid• ESR• CRP• Ferritin• EBV titers• CMV titers• Bartonella titers (if + cat exposure)

• Chest Xray- PA/L• CT scan (CAP)• Lumbar puncture (if NHL)• Bone marrow aspirate/biopsy• PET scan• Least invasive Biopsy

http://www.nejm.org.ezproxy.etsu.edu:2048/doi/pdf/10.1056/NEJMra0904569

Uric AcidPhosphatePotassium

Calcium SodiumNO CHANG

E

Any Questions?