for learning purposes

1. HODGKIN LYMPHOMA
PRESENTOR; VERONICA KANICK, MMED 2
SUPERVISOR; DR HERONIMA PAEDIATRIC ONCOLOGIST

2. Presentation outline
• Case presentation
• Literature review

3. Case summary
• Name: Boniphace Moris Vitalis
• Age: 9 yrs
• Sex: Male
• Residence : Serengeti-Mara
• Informant : Biological mother
C/C
• Abdominal swelling for 1 year and 6/12
• Recurrent fevers 1 year
• Generalized nodular swelling for 6/12

4. HPI:
• He was reported to be apparently well until 1year 6months ago when
he started experiencing abdominal swelling, gradual in onset,
progressively increasing in size, painless, associated a 5 days hx of
passing fresh blood in urine and bleeding per nose which occurred 6
months ago and awareness of HB, easy fatigability and GBM with no
hx of trauma or easy bruising or bleeding from other sites.

5. • 1 year ago he started having fevers, high grade, recurrent
on and off with no specific periodicity, associated with
drenching night sweats however ;
• Denies hx of convulsions, LOC, cough, vomiting, change in
bowel habits, open TB contact or loss of weight.
• No hx of yellowish discolouration of skin or eyes, joint pain
or swelling

6. • 6 months ago he started having nodular swellings which
started on the chest, progressively spread to the face area,
neck, arms, inguinal and LL, progressively increasing in
number
• In the course of this illness the pt have been treated several
times as malaria which was not resolving, received BT 5
times, and was kept on daily folic acid.

7. • PMHX; no hx of previous admissions prior to onset of this illness, no
hx of surgeries, no known allergies
• FSHx; No hx of chronic conditions in the family such as malignancy or
sickle cell disease.

8. O/E; ill looking, febrile (38.7C), dyspnoeic, pale +++, not
cyanosed, not jaundiced, no LL oedema, generalized
lymphadenopathy, matted submandibular and submental,
firm and rubbery, largest on submandibular area measuring
about 5x 4 cm
Vitals; SPO2 98% in RA, PR 110BPM, RR 28CPM
Arthropometric measurement; BWT 26kg, length 127cm,
MUAC= 13cm, BMI= 16 (normal)

9. S/E
P/A: grossly extended, moves with respiration, everted umbilicus, tense
, splenomegaly of 17cm below LCM, tender hepatomegaly of 15cm
BRCM, no shifting dullness, normal bowel sounds
RS: Tachypnoeic, Normal chest cage, multiple subcostal and parasternal
LNs, non tender, symmetrical expansion, bil air entry, no crackles

10. • CVS: tachycardic, S1 and 2 heard with gallop rhythm .
• CNS: normal

11. PDX: 1. lymphoma possibly HL
ddx; EPTB, NHL, leukemias
2.Lymphadenopathic KS
3. PAIDS
4. lymphoproliferative diseases

12. Investigations ;
FBP- pancytopenia
Electrolytes-normal
LFT, RFT- normal
PBS- features of pancytopenia
CSF for cytology
Bone marrow biopsy
Serum Uric Acid
PITC
• Abdominal USS-
hepatosplenomegaly (spleen
18cm, liver 16cm, smooth, no
focal lesions)
• CXR
• CT scan abdomen and chest

13. Treatment
• BT
• Iv ceftriaxone
• Iv PCM

14. Literature review

15. Introduction
• Hodgkin lymphoma (HL) is a malignant process involving the
lymphoreticular system.
• It is an uncommon hematologic malignancy arising from
mature B cells
• Characterized by the presence of Hodgkin cells and reed-
Sternberg cells.
• Account for appr 7% of childhood cancers

16. • Incidence in childhood varies by age
• Very rare in infants
• Most common cancer in the 15-19 year-old age group
• Bimodal age distribution, with peaks at 15-35 yr of age and again
after 50 yr .
• Most common cancer seen in adolescents and young adults,
• Third most common cancer in children younger than the age of 15 y

17. Risk factors for HL
• No clear risk factors , several have been implicated
Infectious agents eg human herpesvirus 6,cytomegalovirus, HIV and
Epstein-Barr virus (EBV)
High socioeconomic status
Family hx
Men >women
Whites >blacks>Asians
Age

18. Pathogenesis
• HL arises in a single node or chain of nodes and spread first
to anatomically contiguous lymphoid tissue.
• Visceral involvement may be sec to extension from adjacent
LNs.
• Hematogenous spread occur to the liver or multiple bone
sites
• Mechanism of spleen involvement is unclear but all patients
with hepatic and bone involvement are a/w splenic
involvement

19. The Reed-Sternberg (RS) cell, a
pathognomonic feature of HL
 It is clonal in origin and arises from the
germinal center B cells but typically has
lost most B-cell gene expression and
function.

20. • HL is characterized by a variable number of RS cells
surrounded by an inflammatory infiltrate of
lymphocytes, plasma cells, and eosinophils in different
proportions, depending on the HL histologic subtype

21. Histologic classification of HL
• WHO classification of lymphomas divides HL int
Classical HL
Nodular lymphocyte predominant HL

22. Classical HL
• Occur more commonly (90-95%) than nodular lymphocyte
predominant HL .
• 4 subtypes;
i. Nodular sclerosis (NS)
ii. Mixed cellularity (MC)
iii. Lymphocyte depleted (LD)
iv. Lymphocyte rich (LR)

23. Nodular lymphocyte predominant HL (NLPHL)
• Accounts for 5%-10% of HL cases.
• Malignant cells seen in NLPHL are called LP cells, aka popcorn cells
Resembles and exploded popcorn
Large
Usually have a single large, vesicular polylobulated nucleus but
distinct and small usually periphery nucleoli without perinuclear
halos.

24. Symptoms and signs of HL
• Common presenting Sxs and signs in children include;
Lymphadenopathy , painless usually cervical, supraclavicular, axillary,
or less often inguinal (rubbery and more firm than inflammatory
adenopathy)
Systemic complaints (B symptoms) fatigue, anorexia, fever, weight
loss >10% in 6mo, drenching night sweats)
Mediastinal mass

25. • Clinically detectable hepatosplenomegaly is rarely encountered.
• Depending on the extent and location of nodal and extranodal disease,
patients may present with
 Symptoms and signs of airway obstruction (dyspnea, hypoxia, cough)
 pleural or pericardial effusion
Hepatocellular dysfunction, or
 Bone marrow infiltration (anaemia, neutropenia, or thrombocytopenia)

26. Diagnosis of HL
• Complete evaluation is important before beginning treatment
in order to evaluate extent of disease to determine the clinical
and pathological stage, treatment modality and prognosis.
• Evaluation includes history, physical examination, and imaging
studies , tissue biopsy and laboratory studies

27. • History ;
Extent and duration of adenopathy
Constitutional sxs eg fever, night sweats, weight loss, previous
infections, immune deficiencies, familial cancer including HL.
• P/E: general health, nutritional status, size and location of LNs,
liver and spleen size, skin infiltrations, pulmonary findings,
neurological signs, tonsils , base of tongue and nasopharynx
(waldeyer’s ring)

28. • Imaging
Goal is to evaluate extent of the disease and guide
tissue biopsy
Chest radiograph (intrathoracic structures,
mediastinal involvement, airway patency)
 CT scans of the neck, chest, abdomen, and pelvis;(
info about extra nodal sites )
Positron emission tomography (PET) scan ( detect
both nodular and diffuse disease, sensitive than
bone marrow to detect BM involvement)

29. Xray showing mediastinal mass

30. • Tissue biopsy
DX of HL is by histologic examination
Excision biopsy of an enlarged LN to demonstrate RS
cells or their variant
Bone marrow aspirate for pts with advanced disease
(stg III and IV), B sxs or any abnormality on FBP

32. • Lab investigations
Performed to investigate organ involvement and propensity
for pt relapse following conventional therapy
CBC
ESR
RFT,LFT, electrolytes
LDH
Urinalysis
Serum ferritin ; prognostic

33. Staging of HL
• Important to guide therapy stratification and prognosis.
• Currently defined by Ann Arbor staging system with
Cotswolds modifications used both in adults and children

35. Treatment;
• Treatment is risk adopted and depends on the stage of the
disease.
• Largely determined by disease stage, presence or absence of
B symptoms, and the presence of bulky nodal disease
• Stg I and II= early stage, stage III and IV= advanced stage
• Chemotherapy and radiation therapy are both effective in
the treatment of HL

36. • Chemotherapy agents commonly used to treat children and
adolescents with HL include;
 cyclophosphamide, procarbazine, vincristine or vinblastine,
prednisone or dexamethasone, doxorubicin, bleomycin,
dacarbazine, etoposide, methotrexate, and cytosine
arabinoside

37. • The combination chemotherapy regimens in current use include;
COPP (cyclophosphamide, vincristine [Oncovin], procarbazine, and
prednisone) or
ABVD (doxorubicin [Adriamycin], bleomycin, vinblastine, and dacarbazine),
with the addition of prednisone, cyclophosphamide, and etoposide (ABVE-
PC and BEACOPP) or
 BAVD (brentuximab vedotin, doxorubicin [Adriamycin], vincristine,
dacarbazine) in various combinations for intermediate- and high-risk
groups

38. Relapse
• Most relapses occur within the 1st 3 yr after diagnosis, but
relapses as late as 10 yr have been reported
• Relapse cannot be predicted accurately with this disease.
• Poor prognostic features include tumor bulk, stage at
diagnosis, extralymphatic disease, and presence of B
symptoms.

39. • Approximately 25% of patients suffer from disease relapse or
are refractory to initial therapy
• Treatment is generally given as an alternative combination
chemotherapy to the initial regimen and, if necessary, with
radiotherapy to sites of bulky disease

40. Prognosis
• The prognosis depends on age, stage and histology.
• Overall approximately 85% of patients are cured.

41. References
• Nelsons text book of paediatrics 20th edition
• Hoffman's essential haematology
• Up to date

42. THANK YOU.