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OROPHARYNX DIFFUSE LARGE B CELL LYMPHOMA

STADIUM II B(E), IN 31 WEEKS MULTIGRAVIDA


PREGNANCY

dr Eriza Sp.T.H.T.B.K.L (K)


Non-Hodgkin Lymphoma (NHL)  Most common
hematological malignancy worldwide accounting for
approximately 3% of the global cancer burden

• WHO 2008  about 40% of the total


NHL cases
• Indonesia  Lymphoma and
Diffuse large leukemia ranked 6th in severity
B-cell lymphoma DLBCL can:
(DLBCL) • Complete remission - after first line
therapy
• Relapse - 30-40%
• Refractory - 10%

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National Data  does not
combine obstetrics and gynecology data
(not actual numbers)

Prevalence of Maggen et al. (2020)  17 from


pregnancy with 100,000 live births and 25 to 27 per
malignancy 100,000 pregnancies

Wolters et al. (2021)  1 in


2000 pregnancies are cancer related

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One case reported pregnancy
Mussiroh dan Bintoro (2023)
with NHL in Indonesia

Case Report
Woman, 27 years old, 31 weeks pregnancy with Oropharynx Diffuse
Large B Cell Lymphoma Stage II B (E)

Interdisciplinary collaboration (obstetrics and gynecology,


pediatrics, ENT, clinical nutritionist, radiotherapy) in establishing
the diagnosis & management of pregnant patients with NHL

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ANAMNESIS (Alloanamnesis)

Chief Complain
• A lump in the neck that has been getting
bigger since 2 months. Patient complained
that there was a lump in the mouth, throat
(+), soarness (+), shortness of breath (+).
Weight loss (+). Another lump is in the
armpit
• Shortness of breath became more severe,
consulted to the ENT department à
tracheostomy operation and oropharynx
tissue biopsy à malignant tumor à IHK
examination.
• The patient turned out to be preterm
pregnancy (29 weeks).

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Physical Examination
Oropharyngeal Region
• A lumpy right tonsillar
mass appears filling
the oropharynx, no
discoloration, active
bleeding (-), posterior
pharynx cannot be
assessed

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Colli and Axilla Regions
• Dextra colli region: Multiple
nodules at level III-IV, size
7x10x5 cm, partly reddish in
color, solid, firm boundaries,
pain (-), fluctuating (-)
• Axillary region: Visible nodule
measuring 2x3 cm, no disco-
loration, solid, firm
boundaries, pain (-),
fluctuating (-)

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Physical Examination

Specific Examination (continued)


Colli Dextra : Multiple nodules appeared at level III-IV, size
7x10x5 cm, partly reddish in color, solid, firm
boundaries, pain (-), fluctuating (-)

Axilla Dextra : A nodule measuring 2x3 cm, no discoloration,


solid, has firm boundaries, pain (-), fluctuating (-)

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SUPPORTING INVESTIGATION

Impression :
• Normal cor and pulmo
• Enlarged right
paratracheal lymph node

Impression :
• There was no visible intra-
abdominal metastatic process
• Gravidarum uterus 29 weeks 4
days

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SUPPORTING INVESTIGATION

Impression:
Malignant tumor of
oropharynx, non-
Hodgkin lymphoma

• Conclusion: Non Hodgkin lymphoma high grade in


accordance with the Diffuse Large B Cell
Lymphoma (DLBCL), Non GCB subtype in
oropharynx (CD20+, KI67 High grade, Mum1+ ,
BCL2+, BCL6+)

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DIAGNOSIS

Oropharynx Diffuse Large B Cell Lymphoma


Stage II (E), Chronic Disease Anaemia and
Severe Malnutrition + Post Tracheostomy e.c
Partial Airway Obstruction in Multigravida 31
Weeks Pregnancy

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INTERVENTION
 NON PHARMACOLOGICAL

High Calories and Protein diet 1900 kcal


Education
Observe vital signs and FHR
Tracheostomy toillete and care
Palliative radiotherapy plan
Chemotherapy plan with the R-CHOP regimen

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Post radiotherapy patient condition

Post Radiotherapy Examination (Colli Region and Oropharynx)

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DEVELOPMENT DURING TREATMENT

Date 5 June 2023


Fetomaternal Ultrasound - AEDV appearance in the umbilical artery
- Notching of the uterine arteries
- 32 weeks pregnancy with head
presentation, asymmetrical PJT
- Fetal maternal hypoperfusion
(decompensated fetus)

Therapy - Termination of pregnancy via section


Secaria conducted at 12.45 WIB
- The baby was born at 13.18 WIB, male,
A/S 3/4, weight 1,170 grams, body length
38 cm, head circumference 29 cm

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DEVELOPMENT DURING TREATMENT

Date 8 June 2023

Picture 17. Patient's Ovarian Organs


Inspection Check the Anatomical Pathology and CPI of the
Plan Sinistra Ovaries

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DEVELOPMENT DURING TREATMENT
Date 13 June 2023
Examination • Cough (+), fever (-), shortness of breath (-)
history • 110/70 mmhg
• 90 x/m
• 20 x/m
• 37,1 C
• 99% with free air
• Rough rhonki at the apex of the
right lung

Airway obstruction reduced

Therapy Paracetamol 3x1 gr IV if needed


Resfar 1x5 gr IV
Cetirizine 1x10 mg
Levofloxacine 1x750 mg IV (H-2)

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Follow Up
• Currently the OS is in good
general condition, the patient
has undergone 4 cycles of
chemotherapy with the RCHOP
regimen.
• The patient has been
decannulated.
• There is still a lump in the neck,
but it is much smaller

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Discussion
In general

Indolen Follicular lymphomas and chronic lymphocytic


LNH leukemia/small
Aggressive LNH. lymphocytic
Diffuse largelymphomas.
B-Cell lymphomas
(DLBCL).
OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)

In case :

 Neck lump that is getting bigger, pain in the


lump (-), does not respond to OAT
administration suspected symptoms of
lymphoma

B Symptoms (last 3 months)


• Fever
• Night sweats
• Weight loss >10%

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OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)

 In Case :

Important findings on physical examination of NHL

Painless enlarged lymph nodes  usually ignored by patient and


makes delayed diagnosis

Cervical Limphadenopaty
The head and neck presentation is most common in NHL 
characterized by multiple painless nodules

Singh R, Shaik S, Negi BS, et al. Non - Hodgkin ’ s lymphoma : A review. Published online 2020. doi:10.4103/jfmpc.jfmpc
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OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)

Lymph node biopsy during pregnancy:


Pathological • Superficial lymph nodes – local
examination of anesthesia
lymph node biopsy • No superficial lymph node -
specimens general anesthesia

Risks of elective surgery


in pregnant women
To confirm the
diagnosis of HL or
NHL Safe even during the first
trimester

Pereg D, Koren G, Lishner M. The treatment of Hodgkin’s and non-Hodgkin’s lymphoma in pregnancy. Published online 2007. doi:10.3324/haematol.11097
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OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)

Helps to straighten up
diagnosis
Radiological
examination of Staging determination
lymphoma
Assessment of disease
response to therapy
Radiation
exposure to the Lower than
fetus in most the threshold It should not
pose a risk to
radiographic dose for
the fetus
examinations adverse effects
(neck and chest on the fetus
x-rays)
Pinnix CC, Andraos TY, Milgrom S, Fanale A, Blvd H, Blvd H. The Management of Lymphoma in the Setting of Pregnancy. 2018;12(3):251-256.
doi:10.1007/s11899-017-0386-x
Lowe S. Diagnostic imaging in pregnancy : Making informed decisions. Published online 2019. doi:10.1177/1753495X19838658
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OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)
Ann Arbor Classification of Hodgkin Lymphoma

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines® ): B-Cell Lymphomas.; 2023. 23
OROPHARYNX DIFFUSE LARGE B CELL
LYMPHOMA (DLBCL)

Check up result Based on size and


histopathological biopsy
number of nodules
• Overview of non-Hodgkin's refer to NCCN
lymphoma (NHL) (2023)

Immunohistochemistry
examination results (CPI)
• CD20 (+), CD3 (-), Extranodal
(Positive approximately 90%
Ki67 Oropharynx
on tumor cells), BCL
2 (+), BCL 6 (+), and Stadium II B
Mum 1 (+)

Impression diffuse large B-Cell lymphoma (DLBCL) Stadium II B

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• Lumps are found in 1 lymph node or a group of nearby lymph
tag nodes

• Lumps are found in 2 or more groups of lymph nodes on


tag the same side of the diaphragm

• Bulky disease means there is an area of lymphoma measuring


tag 7.5 centimeters (cm) or larger.
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• Lumps found in the lymph nodes above and below the
tag diaphragm on the same side of the body or disease found in
the lymph nodes above the diaphragm and in the spleen.

tag • The lump has spread outside the lymphatic system to other parts
of the body.
How is pregnancy managed in this case?

• Lymphoma management:
Aims to treat combination of
adequately without chemotherapy and multi-
harming the fetus. agent radiotherapy.

Multidisciplinary Radiotherapy to local


disease areas: early
approach stage treatment
modality/additional
chemotherapy in high
risk
CHOP-like

diffuse large B-Cell lymphoma (DLBCL)

• Premature birth
• Disease-related small gestational age (SGA) neonates
• Prematurity-related neonatal complications are
common

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Chemotherapy Radiotherapy
• Delay labor for 2-3 weeks after • The threshold dose for exposure to
treatment to allow bone marrow severe congenital malformations when
recovery administered during the organogenesis
stage (first trimester) of 0.1-0.2 Gy.

• Premature babies have capacity • Radiation exposure in the second


limited to metabolize and eliminate and third trimesters is associated
drugs because the liver and kidneys are with carcinogenic effects
not yet perfectly developed
• Disease-free survival using radiation
therapy alone is only 60% to 70% in
5 years
PREGNANCY MANAGEMENT IN CASES

The CPI shows the results of CD20 (+), CD3 (-), Ki67 (Positive more or less
90% in tumor cells), BCL 2 (+), BCL 6 (+), and Mum 1 (+)

DLBCL with CD20+


RCHOP

Radiotherapy is required multidisciplinary at low doses


EFFECTS OF MATERNAL CLINICAL
CONDITIONS ON THE FETUS

Patients in this case may be planned for


radiotherapy

Radiotherapy on Radiotherapy on second and


first trimester third trimesters
• Increases the risk of • Increased risk of
congenital malformations developing leukemia and
• It is estimated to occur at tumors in the first decade
of life
doses exceeding the
threshold dose of 0.1-0.2
Gy

It is necessary to consider the risks and benefits of giving


radiotherapy
Maggen C, Dierickx D, Cardonick E, et al. Maternal and neonatal outcomes in 80 patients diagnosed with non-Hodgkin lymphoma during pregnancy: results from the International Network
of
Cancer, Infertility and Pregnancy. Br J Haematol. 2021;193(1):52-62. doi:https://doi.org/10.1111/bjh.17103
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Cesarean section?
If chemotherapy has just been given or the next cycle is planned, a
caesarean section is recommended to be avoided

Inhibition of cell metabolism, cell


division, and angiogenesis

Adequate wound care such as


Negative wound pressure therapy (NPWT)
Wound Dehiscence post chemotherapy

Pervaginal Labour ?
There is no data that explains that vaginal delivery is contraindicated
unless there is an obstetric indication for perabdominal delivery
What is the effect of the maternal clinical
condition on the fetus in this case?

Fetal exposure In NHL, there are


to several cases that Preterm labor (52%),
chemotherapy is documented with obstetric
associated with good maternal and complications (41%),
an increased fetal outcomes neonatal (12.5%) and
risk of FGR despite exposure SGA neonates (39%)

Premature birth is avoided as much as possible unless it is


due to maternal factors.

In patients with advanced cancer and systemic burden or


inappropriate treatment  preterm birth may be considered
Irwinda R. Panduan Anemia dalam Kehamilan. In: 23 Rd Annual meeting indonesian society of maternal fetal medicine panduan anemia dalam kehamilan. ; 2023., National Comprehensive Cancer Network. Diffuse large b-cell lymphomas. Natl Compr Cancer Netw, National Comprehensive
Cancer Network. NCCN clinical practice guidelines in oncology (NCCN Guidelines® ): B-Cell Lymphomas.; 2023., Soeters PB, Wolfe RR, Shenkin A. Hypoalbuminemia: pathogenesis and clinical significance. J Parenter Enter Nutr. 2019;43(2):181-193. doi:10.1002/jpen.1451
Conclusion
• The importance of interdisciplinary collaboration
(obstetrics and gynecology, paediatrics, ENT,
clinical nutritionist, radiotherapy) in establishing
the diagnosis & management of pregnant
patients with NHL. In this case, the patient was
treated with radiotherapy, then after the patient
gave birth, RCHOP chemotherapy was given and
the patient's outcome is currently showing good
progress.

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