Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 14, Number 1, January 2023, pages 36-43


Uncommon Presentation of Undiagnosed B-Cell Lymphoproliferative Disorder as Nodular Pulmonary Amyloidosis

Figures

Figure 1.
Figure 1. Chest X-ray showed extensive pulmonary nodulo- reticular shadowing at the peripheries signifying extensive fibrotic changes with scattered nodules.
Figure 2.
Figure 2. CT chest showed several nodules bilaterally: a calcified nodule (1.4 cm) in the apical segment of the left lower lobe and a 2-cm calcified nodule along the posterior mediastinal pleural surface of the left lung (blue arrow), above the level of the aortic knob. CT: computed tomography.
Figure 3.
Figure 3. (a) Low magnification H&E stain. (b) High magnification H&E stain showing homogenous acellular amorphous material (amyloid) with plasma cells. (c) High magnification H&E stain showing homogenous acellular amorphous material (amyloid) with plasma cells. H&E: hematoxylin and eosin.
Figure 4.
Figure 4. (a) Unpolarized Congo red stain. (b) Polarized Congo red stain showing apple-green birefringence.
Figure 5.
Figure 5. Immunohistochemistry showing kappa light chains.
Figure 6.
Figure 6. Immunohistochemistry showing lambda light chains.

Table

Table 1. A Comprehensive Review of Similar Case Reports Published by Other Authors
 
Study name and yearAge (years)/sex (M or F)Presenting featuresPrimary tumorImmunostaining profile in addition to pathologic diagnosis of amyloidosisTreatmentOutcome
F: female; M: male; H. pylori: Helicobacter pylori; +: positive; MALT: mucosa-associated lymphoid tissue; RLL: right lower lobe; PMH: past medical history; CXR: chest X-ray; AL: immunoglobulin light chain amyloidosis; IgH: immunoglobulin heavy chain; UV: ultraviolet; CCL: centrocyte-like.
Core et al, 2017 [22]69/FProgressive dyspnea, hypoxemia, dry cough, weight loss, bilateral crackles and wheezes upon auscultation, + respiratory distressExtranodal marginal zone B cell lymphoma of MALT type with bilateral nodular pulmonary amyloidosisLymphocytic cells (CD20+) with scattered atypical plasma cells (kappa+, lambda+, CD138+)RLL wedge resection by video-assisted thoracoscopic surgery, bortezomib, cyclophosphamide, dexamethasone, glucocorticoids, rituximabImproved in the first 6 months; later worsened with acute on chronic respiratory failure
Xiang et al, 2015 [23]59/MAsymptomaticPulmonary MALT lymphoma of the left lower lobeLymphocytes (+ for CD79a, CD20, CD138, bcl-2, lambda light chain, CD43)Left lower lobectomy with mediastinal lymphadenectomyNo recurrence or metastasis reported 14 months after initial diagnosis
Filippi et al, 2019 [24]50/MHemoptysisPrimary pulmonary MALT lymphoma with pulmonary amyloidosisB cells (+ for CD79a, CD20, and lambda light chain)Left upper lobectomyNo local recurrence or metastasis in 2-year follow-up period
Moriyama et al, 2000 [25]63/FAsymptomaticMALT lymphoma of the anterior mediastinum (thymus) with nodular pulmonary amyloidosis of the lungLobular structures within Hassall’s corpuscles (keratin+). Few plasma cells (+ for IgG and kappa light chain) along with medium CCL cells (B cell phenotype + for CD20 and CD79a)Complete thymus resection by sternotomy, resection of lung nodules by left thoracotomyNo recurrence of lymphoma in 47-month follow-up period
Lantuejoul et al, 2007 [26]50/FAsymptomatic with PMH of MALT-type gastric lymphoma due to H. pylori. CXR showed incidental multiple pulmonary nodules.MALT-type marginal zone lymphoma involving gastrointestinal tract, bone marrow and lungs (stage IV)Giant cells, crushed plasma cells with thioflavin T staining under UV light, small plasmacytoid lymphocytes, and large lymphocytes (CD20+)Surgical resection of pulmonary nodules with adjuvant chemotherapy (rituximab), and bone marrow transplantationNo change in nodules and cysts on radiography during the 17-month follow-up period
Davis et al, 1991 [27]56/MLeft upper chest pain, hemoptysis, significant smoking history, wheezes over the left upper chest upon auscultationSmall cell non-Hodgkin’s left pulmonary lymphoma with bilateral lung nodules (nodular pulmonary amyloidosis)Lymphocytic foci with plasma cells, and giant cells (+ for leukocyte common antigen). Both the lymphoma and the amyloidomas showed cells with kappa light chains.Thoracotomy and wedge resection of two left upper lung lobe nodules, prednisone, chlorambucilHilar mass and amyloid pulmonary nodules decreased in size on radiography during follow-up
Wieker et al, 2002 [28]63/FSignificant PMH of MALT-type lymphoma of right upper eyelidMALT-type lymphoma (low grade) of the right upper eyelid with AL amyloid depositsLymphocytic cell infiltrated with plasma cells (+ for Ki-B3, CD20, CD79a, IgM and h-light chain)Cyclophosphamide, doxorubicin, hydroxydaunorubicin, oncovin and prednisoneNo change in number and size of the pulmonary nodules at 6 months and 18 months follow-up
Kawashima et al, 2005 [29]75/FAsymptomaticNon-Hodgkin’s primary pulmonary MALT-type lymphoma of the right lung with nodular pulmonary amyloidosisB lymphocytes (+ for L26 and CD79)Partial resection and enucleation of right middle lobe nodules with lobectomy of the right middle and lower lobesNo recurrence of MALT lymphoma at 4-year follow-up
Ihling et al, 1996 [30]67/MAsymptomatic with PMH of lymphoplasmacytic immunocytoma in the lung and an abnormal lymphoid infiltrate in the bone marrowPrimary extranodal lymphoplasmacytic immunocytomas of the lung or the bronchus-associated lymphatic tissue with intrafocal tumor-like amyloidosisPlasma cells between amyloid deposits (+ for lambda light chains), lymphocytes and plasma cells surrounding amyloid tumor (+ for kappa and lambda light chains), amplification of the IgH gene with sharp bandsNo treatment as hematopoiesis was not affected-
Ihling et al. 1996 [30]66/MAsymptomatic with tumorous growths in the right lower and left lower lobes on chest X-rayPrimary extranodal lymphoplasmacytic immunocytomas of the lung or the bronchus-associated lymphatic tissue with intrafocal tumor-like amyloidosisPlasma cells (+ for lambda and kappa light chains in equal frequency), lymphocytes from lung biopsy (CD20-reactive B-lymphocytes and CD3-reactive T-lymphocytes), amplification of the IgH with sharp bandsRemoval of right lower lobe and mediastinal lymphadenectomy-