Diversity of Radiological Imaging and Clinical Course in Pulmonary MALT Lymphoma by Gülfidan Aras in Journal of Clinical Case Reports Medical Images and Health Sciences.

Diversity of Radiological Imaging and Clinical Course in Pulmonary MALT Lymphoma by Gülfidan Aras in Journal of Clinical Case Reports Medical Images and Health Sciences.

 

Abstract

A 59-year-old male patient was admitted to the emergency department with
a three-month history of worsening dyspnea, fatigue, and cough. His vital
signs were recorded as follows: blood pressure 138/88 mmHg, heart rate 98
beats/min, respiratory rate 25 breaths/min, temperature 37.8°C, and oxygen
saturation 91%. During the lung auscultation, breath sounds were absent in the
lower left lung, while crepitant rales were audible in the upper zone and the
right lung. CT scan of the chest showed 1 cm lymph nodes, pleural effusion,
fibrotic changes, varicose-cystic bronchiectasis, as well as consolidations
and atelectasis with air bronchograms in both lungs. Furthermore, thoracic
ultrasonography revealed a large effusion in the left hemithorax, measuring 11
cm. He was hospitalized after the placement of a pleural catheter. Radiological
diversity and the clinical course of the patient posed challenges for establishing
a differential diagnosis.
TL; DR: In this paper; we aimed to present a case of MALT lymphoma that
manifested in the lung and caused diagnostic confusion with radiological and
clinical symptoms.
Introduction
MALT (Mucosa-Associated Lymphoid Tissue) is the lymphoid tissue that
plays a role in mucosal defense. It includes functional memory B lymphocytes,
which are essential for the immune response. They are not physiologically
present in the lungs, but they become active there in response to infections
and chronic antigenic stimulation. Marginal zone B-cell non-Hodgkin lymphoma
(MALT Lymphoma) accounts for 8% of adult lymphomas. Although it is most
commonly seen in the stomach, it can also be seen in the salivary glands, thyroid
and lungs (1).
It is the most common type of lymphoma in the lung. It presents common
radiological, pathological, and clinical findings with infection and other
granulomatous diseases, making differential diagnosis quite difficult for the
clinician. In this paper, we aimed to present our case, which we had difficulty in
diagnosing in the clinic.
Case Presentation
A 59-year-old male patient was admitted to the emergency department
with worsening dyspnea, fatigue, and cough over the past three months. The
chest X-ray of the patient showed a consolidation extending from the center
to the periphery in the left upper zone near the aortic arch, an increased
density indicative of pleural effusion with sinus obliteration on the left, and a
consolidation extending from the hilum to the lower zone near the heart

During the lung auscultation, breath sounds were absent
in the lower left lung, while crepitant rales were detected in
the upper zone and on the right side. Blood pressure was
138/88 mmHg, pulse was 98 beats /min, respiratory rate
was 34 breaths/min, and oxygen saturation was 91%.
At the time of admission, the patient's biochemical
analysis results were as follows: Glucose 77 mg/dL (normal
range: 70-115), urea 31 mg/dL (normal range: 17-43),
creatinine 0.77 mg/dL, protein 65.8 g/L (normal range: 66-
83), albumin 39.3 g/dL (normal range: 35-53), LDH 423 U/L
(normal range: <247), CRP 21.6 mg/L, procalcitonin <0.01

lymphocyte dominance in the cell count. In the biochemical
analysis, the pH was 7.440, lactate dehydrogenase (LDH)
was 206 U/L, total protein was 39.40 g/dL, albumin was
25 g/dL, glucose was 60 mg/dL, and adenosine deaminase
(ADA) was 34.4 U/L. Gram staining of the fluid, bacterial,
fungal, and acid-fast bacilli growth were all negative. The
patient's fluid was drained by aspiration. An intrapleural
catheter was placed due to the high amount of fluid and
increased dyspnea. The patient was admitted to the
ward and initiated on oxygen therapy, bronchodilators, and
antibiotics. The pleural fluid sent for cytological analysis two
more times during the patient's hospitalization was found to
be serohemorrhagic. Upon reevaluating his microbiological
results, no growth was observed. Although many lymphoid
cells were seen in the cytopathological examination of the
patient's second pleural fluid, no atypical features were
monitored. The patient's condition stabilized during clinical
follow-up, and the pleural catheter was removed. However,
after a while, the patient's dyspnea complaint recurred and
the catheter was placed again because of the increase in
fluid on the radiograph (Figure 3). There was no change in
the infection markers of the patient, who also had fever from
time to time, and CRP ranged between 20 and 16 mg/dL
during follow-up. There was no growth in his small amount
of sputum and blood cultures taken during the fever.
The patient underwent fiberoptic bronchoscopy and
no endobronchial lesions were detected. Wang fine needle
aspiration and bronchial lavage were applied to mediastinal
lymphadenomegaly Wang IA revealed lymphoid cells, but a
definitive diagnosis could not be obtained. No findings were
found in the lavage other than bronchial epithelial cells and
polymorphonuclear leukocytes

Pathology:
Samples taken from the parietal pleura and the nodule
on the diaphragm were evaluated as low-grade non-Hodgkin
Lymphoma and interpreted as extra-nodal marginal zone
lymphoma (MALT) by the pathologist (Figure 5).
Discussion
Clinical:
The patient, who had been admitted to the emergency
room with symptoms of dyspnea, hypoxia, and fever, was
hospitalized after pleural fluid was exudate and consolidation
was detected. Although the patient's clinical symptoms were
severe, CRP was moderately high, and the occasional fever
despite antibiotic therapy during hospitalization suggested
diagnoses such as malignancy and tuberculosis, in addition
to non-specific infection.
Lymphomatous proliferation can involve the lung in
various ways. Non-Hodgkin or Hodgkin lymphoma can
present in the lung through hematogenous spread or by
invading from adjacent mediastinal lymph nodes. However,
primary involvement of the lung is also possible. Primary
lymphomas should not have extra-pulmonary organ
involvement for at least 3 months after diagnosis. The most
common are MALT and effusion lymphomas (2).
Effusion lymphomas may involve the pericardial
and peritoneal cavities, with the most common primary
involvement being the pleura, without solid organ
involvement. Human-Herpes-8 infection and EBV may be
accompanied by fever and lymphocytic-exudative fluid.
HHV-8 negative cases have a better prognosis (3).
In this case, there was also an exudative pleural effusion.
However, although pleural involvement was not detected
in the fluid cytological examination, pleural involvement
was detected in biopsies. However, it is not possible to say
that the patient only has effusion lymphoma (PEL). MALT
(Mucosa-associated Lymphoid Tissue) lymphoma is the
type of lymphoma that most commonly involves pulmonary
tissue, is often asymptomatic, and shows radiological
alveolar opacities. Although it is more commonly affected
in people aged 50-60, it can rarely be seen under the age of
30. It constitutes 60% of pulmonary lymphomas. Weight loss
and fever are especially prominent during the aggressive
phase, though the condition may initially be asymptomatic.
Autoimmune disease may be the basis in 16% of cases
(1,4). The prognosis of MALT lymphomas is good; 5–10-year
survival is more than 80% (5).

atypical structures were observed. There were findings of
granulomatous inflammation in the samples obtained from
the mediastinal lymph nodes of the patient. In fact, in the
biopsy samples taken by video-assisted thoracoscopy, low-
grade B-cell non-Hodgkin Lymphoma (CD20 positive (diffuse
cells), anti-BCL-2 positive, anti Ki-67 low positive) and extra-
nodal marginal zone lymphoma were detected in the parietal
pleura. Pathologically, the lymphomatous infiltrate in MALT
lymphoma exhibits heterogeneous features and consists
of small lymphocytes, centrocyte-like cells, monocytoid B
cells, rarely large transformed cells and plasma cells (10).
Necrosis is rare. Neoplastic cells are expressed in CD 20 and
CD 79. Ki67 index is lower than 20%.
Lymphoma and sarcoidosis are similar in terms of
clinical and radiological phenotype. Distinguishing low-
grade lymphomas from sarcoid lesions can be challenging;
sarcoid granulomatous lesions may be accompanied by
lymphoid cell infiltration. In addition, sarcoid-like reactions
are frequently seen in malignant lymphomas. Moreover,
sarcoidosis-lymphoma syndrome was first described in
the study by Brincker et al. They reported that lymphoma
was 5.5 times more common in sarcoidosis patients than
in the general population, indicating the presence of
sarcoidosis years before lymphoma (11, 12). Kokuho N et
al reported MALT lymphoma in the lung for the first time in
a ten-year-old sarcoidosis case with ocular, gastric and lung
involvement (13).
In this case, granulomatous inflammation was detected
in mediastinal LAMs, but no findings of sarcoidosis were
found in ocular, cardiac, renal examinations, angiotensin-
converting enzyme, calcium, alkaline phosphatase, and 24-
hour urine calcium analyses. There was also no abnormality
in abdominal ultrasonography. We believe that the
granulomatous inflammation in our case was reactive to
immune deficiency.
Conclusion
The patient was started on Rituximab treatment by
the hematology department. Clinical and radiological
improvement was observed following treatment. In the
follow-up PET-CT examination, regression in consolidated
areas, decrease in FDG uptake, and metabolic partial
regression were detected compared to the initial
examination (Figure 6). Lymphomas, which do not have
specific radiological and symptomatic features, can mimic
most diseases of the respiratory system and do not present
with a noisy picture, requiring the clinician to be persistent.

For Information:JCRMHS