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Case Report: Burkitt Lymphoma

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CASE REPORT: BURKITT LYMPHOMA

Estudo de Caso: Linfoma de Burkitt

Amanda Fernandes Vieira1

Débora Magalhães Paiva2 Emanuelly Durães Rocha3 Matheus Cardoso Murta Botelho3 Thaisa Silva Lima3 Sabrina Araujo Gomes Cabral4

Abstract: This article analyzes the case of Burkitt lymphoma, an unusual type of lymphoma, which is

very aggressive in its reactions, originating from the immune system cells, having as biological mark, the

translocation involves immunoglobulin and c – MYC genes. Of the patients affected by Burkitt lymphoma,

half of them are under the age of 40 and one third affect people between the ages of 15 and 40. The study

also analyzes the case of a 25-year-old male patient who was admitted to Santa Casa de Montes Claros

Hospital in January 2015, diagnosed with high-grade B lymphoma. The study therefore addresses all the

Burkitt lymphoma characteristics that have developed in this patient, as well as the clinical and therapeutic

discussions of lymphoma. It also presents the numerous and frequent intercurrences presented during the

progression of the disease and during the treatment itself.

Keywords: Lymphoma; Patients; Disease.

________________

Corresponding author: Amanda Fernandes Vieira. E-mail: emanuelly.rocha.def@hotmail.com 1 Faculdades Integradas do Norte de Minas. 2 Universidade Estadual de Montes Claros.

3 Faculdades Integradas Pitágoras de Montes Claros. 4 Hospital Santa Casa de Montes Claros.

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REVISTA UNIMONTES CIENTÍFICA

Montes Claros, I Congresso Nacional de Oncologia da Associação Presente - Agosto de 2017.

273

Resumo: O presente artigo faz uma análise e estudo de caso acerca do linfoma Burkitt, sendo este um tipo

incomum de linfoma, bastante agressivo em suas reações, se originando de células do sistema imunitário,

tendo como marca biológica a translocação envolvendo imunoglobina e genes c- MYC. Dos pacientes

acometidos com o linfoma Burkitt metade são menores de 40 anos e um terço atinge pessoas entre 15 e

40 anos. O estudo faz ainda uma análise do caso de um paciente do sexo masculino com idade de 25 anos,

que deu entrada no Hospital Santa Casa de Montes Claros em janeiro de 2015, com diagnóstico de linfoma

B de alto grau. O estudo aborda, portanto todas as características do linfoma Burkitt, que desenvolveram

neste paciente, bem como as discussões clinicas e terapêuticas do linfoma. Apresenta ainda as inúmeras e

freqüentes intercorrências apresentadas durante a evolução da doença e durante o próprio tratamento.

Palavras-chave: Linfoma; Pacientes; Doença.

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REVISTA UNIMONTES CIENTÍFICA

INTRODUCTION

Lymphomas are a group of neoplastic

diseases that originate from immune system

cells, and specifically affect the cells from the

lymphopoietic system, which is responsible for

producing the lymphocytes. Their classification

depends on the lineage of cells that are developed.

The majority affects the lymphocytes type B, and

to a lesser extent T cells and natural killer (NK).

1

Burkitt’s lymphoma (LB) is an uncommon

variant, constitutes less than 1% of all

non-Hodgkin lymphomas.

2.3

Its biological mark

is a translocation involving immunoglobulin

and c-MYC genes. Of the patients affected by

Burkitt’s lymphoma half of them are under 40

years old and one third affects people between 15

and 40 years old.

There are three subtypes, the first is known

as endemic, affects children and adolescents,

especially on the African continent, it is related in

95% of cases with the Epstein-Barr Virus (EBV).

The sporadic subtype does not present a defined

geographic distribution, EBV is only confirmed

between 10 and 20% of cases.

5

The sporadic

subtype affects typically the ileocecal region,

intestinal walls and mesentery. The third subtype

affects immunocompromised patients infected

by the Human Immunodeficiency Virus (HIV).

It is one of the tumors of faster growth known,

is highly aggressive even though it has good

response to treatments and is potentially curable.

1

The prognosis of LB patients is

significantly related to age, with increased rate of

curability in pediatric patients in comparison with

adults, with worsening even higher in elderly.

4

The objective of this study reports the

case of a patient with Burkitt’s lymphoma,

non-Hodgkin of cells B.

CASE REPORT

Patient R.V.M, male , 25 years old,

brown, was admitted to the oncology service

of the Hospital Santa Casa de Montes Claros

on January 12th, 2015, after confirmation of

diagnosis of lymphoma B of high degree by

means of Immuno-histochemistry (Ki-67,M1B1

+;CD10,S6C6 +; CD20, L26 +; CD3 - polyclonal

) . It was reported in this period that the patient

had epigastric pain, fullness, constipation and

weight loss of five kilos for 45 days.

At the physical examination, the patient

was hydrated, without adenomegaly, eupneic,

normal heart sound hypophonetic in two different

times (BNRNF2T), palpable spleen 6 cm from

the left costal edge. Vital Signals: B.P:

120x80

mmHg,

H.R

76Bpm, R.R

:

18 Irpm, Axillary

temperature

:

37 °C. Laboratory exams: LDH

:

1373; VHS

:

72; Hb

:

10,6g/dl; LC

:

13630 N

:

68

B

:

15L 10; Platelets:

214.000; creatinine

:

0,82;

GPT

:

67; GOT

:

46. Serologies for HIV,

anti-HCV and anti-HBC negative.

On admission, imaging studies were

carried out, such as computed tomography (CT)

of the chest which showed amorphous tissue

formation, in left paracardiac situation; laminar

bilateral pleural effusion to the right, and small

to the left, noting outbreaks of consolidation/

atelectasis of the lung segments adjacent to that

side.

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REVISTA UNIMONTES CIENTÍFICA

Montes Claros, I Congresso Nacional de Oncologia da Associação Presente - Agosto de 2017.

275

parietal diffuse and asymmetric thickening of the

stomach with involvement of fundus, body and

antrum, without obstructive symptoms associated

with and without plans of cleavage with head and

body of the pancreas. It was identified extensive

amorphous tissue formation, expansive aspect ,

adjacent to the body, gastric fundus. This tissue,

amorphous formation of 13.9 cm was identified

in the perisplenic region, extending to the left

suprarenal gland. The same lesion was observed

of 6.4cm in the hepatic hilum. The ultrasound

showed splenomegaly.

Chemotherapy was started 15 days after

the admission into the hospital based on two

schemes. In the first scheme, Genuxal®, Mesna®,

Vinciziin®, Adriblastina®, corticoidex® and

Mabthera® were used in cycles I, III, V, VII. In the

second scheme, Methotrexate®, Leucovorin®,

Aracytin®, Soluble-medrol®, MabThera® were

performed , in cycles II, IV, VI, VIII; He was

discharged on January 29th 2015.

Right after the discharge presented

with abdominal pain, of sudden onset, followed

by vomiting and required hospitalization and

subsequently a surgery. In the immediate

postoperative period, he presented febrile

neutropenia with need for Empiric Antimicrobial

Therapy with metronidazole and Ceftriaxona.

After 11 days, it progressed to pneumonia and

moderate left pleural effusion, consolidation

in left basis with air bronchogram. It was used

Vancomycin, Metronidazol and Meropenem. He

was discharged on February 15th.

He returned after twenty three days,

with clinical signals of febrile neutropenia, left

pleural effusion and sepsis in pulmonary focus,

and for treatment vancomycin was used. A week

after, he developed pancytopenia, drug-induced

skin disorder and mucositis as allergic reaction

to chemotherapy. Patient does not evolve with

improvement, he had acute respiratory failure,

requiring a tracheal intubation, evolving with

stability of the clinical signals.

Along the progression he presented signals

of pancytopenia, febrile neutropenia and pleural

effusion, which were repeated in subsequent

hospitalizations. Patient is in remission status,

confirmed by PET-scan with negative residual

lesions.

DISCUSSION

The patient R.V.M exhibited Burkitt’s

lymphoma with sporadic subtype, which has

no specific geographical distribution. It affects

normally the abdominal cavity, as occurred with

the patient.

4

The typical profile of sporadic LB

has massive abdominal presentation and ascites,

involving the distal ileum, stomach, cecum and/

or mesentery, kidney, testicles and ovary, breast

and bone marrow (20%) and the central nervous

system (CNS,14%).

The onset symptoms may be related

to intestinal obstruction or gastrointestinal

bleeding, mimicking acute appendicitis or

intussusception.

The lymphadenopathy, if

present, is usually located.

2.6

Abdominal masses

and hepatosplenomegaly can also occur. Any

sign of lesion of cranial nerve or suspected

of involvement of the CNS motivate new

investigations (examination of the cerebrospinal

fluid (CSF), magnetic resonance imaging and/or

computerized tomography of the brain and spinal

column, depending on the clinical symptoms).

4

(5)

lesions, as explained in the report.

9

The use of anti-CD20, Rituximab,

resulted in improvements on prognosis, bringing

a median survival of 3 years in approximately

90% of young adults (under 55 years).

10

In

adults, Rituximab added to the Hyper-CVAD

(cyclophosphamide, vincristine, doxorubicin and

Dexamethasone alternating with HD-MTX and

Cytarabine).

1

R.V.M of the regime hyper-CVAD

with Rituximab.

FINAL CONSIDERATIONS

The report and publications brings to

light the clinical and therapeutic discussion about

Burkitt’s lymphoma, a neoplasm of infrequent

affection with numerous complications presented

during the progression of the disease. The biopsy

and immunohistochemical analysis are essential

for the definitive diagnosis of the pathology.

Despite the high aggressiveness of the tumor,

treatment with chemotherapy regimen provides a

good prognosis to the patient.

REFERENCES

1. BELLAN C, Lazzi S, Hummel M, et al.

Immunoglobulin gene analysis reveals 2

distinct cells of origin for EBV-positive and

EBV-negative Burkitt lymphomas. Blood.

2005;106(3):1031–1036.

2. BOMKAMM GW. Epstein-Barr virus and

its role in the pathogenesis of Burkitt’s

lymphoma: An un resolved issue. Seminars in

Cancer Biology 2009; 19(6): 351-365

3. CHAN, Onyee et al. “Burkitt Lymphoma

Presenting as an Intracardiac Mass: Case Report

early satiety, constipation, splenomegaly,

pancytopenia and neutropenia.

The diagnosis of LB is based on performance

of biopsy, with immunohistochemistry (HI). At

the IH the neoplastic cells are B cells mature,

more similar to the central germ cell tumors

than activated B-cells.

They express IGM of

monotypical surface CD19, CD20, CD79A,

PAX5 and CD43, besides the antigen of plasmatic

cells CD38 and antigens of germinal center CD10

and BCL6, with a proliferative fraction of KI-67

> 95%.

7

The patient had proliferation of medium

lymphoid cells of blastic aspect confirmed by

immune-hystochemistry with expression of

CD20 and CD10. Fact that demonstrated high

level of cell proliferation compatible with the

findings of B lymphoma of high degree, Burkitt’s

lymphoma .

The laboratory tests include complete

blood counts, renal and hepatic function

tests, including creatinine clearance, level of

calcium and urate, level of LDH in serum and

coagulation. Tests for HIV and hepatitis B and

C are recommended. The instrumental exams

must be carried out quickly so as not to delay the

beginning of chemotherapy and should include a

computed tomography scan of the chest, abdomen

and pelvis more positron-emission tomography

(PET). A bone marrow biopsy is performed to

detect any degree of involvement of the spinal

cord, along with a lumbar puncture medicated

early to cytological diagnosis and analysis

of flow cytometry of CSF.

8

Patient underwent

laboratory tests in the act of admission without

changes,LDH, creatinine,VHS, blood test, GPT,

GOT and with negative serology for anti-HIV,

anti-HCV antibodies and anti-HBC. Currently,

a complete response to treatment is confirmed

with results of PET scan, with negative residual

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Case Report: Burkitt Lymphoma VIEIRA, A. F.; PAIVA , D. M.; ROCHA, E. D.; BOTELHO, M. C. M.;

LIMA, T. S.; CABRAL, S. A. G. ISSN 2236-5257

277

REVISTA UNIMONTES CIENTÍFICA

and Review of Literature.” The American

Journal of Case Reports17 (2016): 553–558.

PMC. Web. 12 July 2017

4. CHESON BD, Pfistner B, Juweid ME, et

al. Revised response criteria for malignant

lymphoma. J Clin Oncol. 2007;25(5):579-586.

5. CIPHAS, Maureen Arboine. “Linfoma de

burkitt: a propósito de un caso.” Medicina

Legal de Costa Rica 34.1 (2017): 325-231.

6. DOZZO, Massimo et al. “Burkitt Lymphoma in

Adolescents and Young Adults: Management

Challenges.” Adolescent Health, Medicine

and Therapeutics 8 (2017): 11-29. PMC. Web.

11 July 2017.

7. GASCOYNE RD, Magrath IT, Sehn L. Burkitt

lymphoma. In: Armitage JO, Mauch PM,

Harris NL, Coiffier B, Dalla-Favera R,

editors. Non-Hodgkin Lymphomas. 2nd ed.

8. HONG J, Kim SJ, Ahn JS, et al. Treatment

outcomes of rituximab plus hyper-CVAD

in Korean patients with sporadic Burkitt or

Burkitt-like lymphoma: results of a multicenter

analysis. Cancer Res Treat. 2015;47(2):173–

181

9. JAIN P, Kantarjian HM, Cortes JE, et al.

Long term follow-up of de novo or minimally

treated Burkitt lymphoma/leukemia

(BL/B-ALL) after frontline therapy per the

hyper-CVAD regimen with or without rituximab:

20-year cumulative experience. Blood.

2013;122:3917.

10. PHILADELPHIA, PA: Wolters Kluwer and

Lippincott Williams & Wilkins; 2010. pp.

334–357.

11. WELEDJI, Elroy P, Marcelin N Ngowe,

and John S Abba. “Burkitt’s Lymphoma

Masquerading as Appendicitis – Two Case

Reports and Review of the Literature.” World

Journal of Surgical Oncology 12 (2014): 187.

PMC. Web. 12 July 2017

12. WILSON WH, Bromberg JE, Stetler-Stevenson

M, et al. Detection and outcome of occult

Leptomeningeal disease in diffuse large

B-cell lymphoma and Burkitt lymphoma.

Haematologica. 2014;99(7):1228-1235.

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