Amyloidosis is a rare metabolic disorder primarily brought on by misfolding of an autologous protein, which causes its local or systemic deposition in an aberrant fibrillar form. It is quite rare for pulmonary tissue to be impacted by amyloidosis; of the three forms it can take when involving pulmonary tissue, nodular pulmonary amyloidosis is the most uncommon. Nodular pulmonary amyloidosis rarely induces clinical symptoms, and most often, it is discovered accidentally during an autopsy or via imaging techniques. Only one case of nodular pulmonary amyloidosis, which manifested as a spontaneous pneumothorax, was found in the literature. In terms of more precise subtyping, nodular amyloidosis is typically AL or mixed AL/AH type. No publications on AH-dominant type of nodular amyloidosis were found in the literature. We present a case of an 81 years-old male with nodular pulmonary AH-dominant type amyloidosis who presented with spontaneous pneumothorax. For a deeper understanding of the subject, this study also provides a review of the literature on cases with nodular pulmonary amyloidosis in relation to precise amyloid fibril subtyping. Since it is often a difficult process, accurate amyloid type identification is rarely accomplished. However, this information is very helpful for identifying the underlying disease process (if any) and outlining the subsequent diagnostic and treatment steps. Even so, it is crucial to be aware of this unit and make sure it is taken into consideration when making a differential diagnosis of pulmonary lesions.

Department of Clinical and Molecular Pathology Faculty of Medicine and Dentistry Palacký University Olomouc Czechia

Department of Clinical and Molecular Pathology University Hospital Olomouc Olomouc Czechia

Department of Hematooncology Faculty of Medicine University of Ostrava Ostrava Czechia

Department of Hematooncology University Hospital Ostrava Ostrava Czechia

Department of Pathology EUC Laboratoře CGB a s Ostrava Czechia

Department of Pathology Znojmo Hospital Znojmo Czechia

Department of Surgery University Hospital Ostrava Ostrava Czechia

Department of Surgical Studies Faculty of Medicine University of Ostrava Ostrava Czechia

Institute for Molecular and Translational Medicine Faculty of Medicine and Dentistry Palacky University Olomouc Olomouc Czechia

Institute of Molecular and Clinical Pathology and Medical Genetics Faculty of Medicine University of Ostrava Ostrava Czechia

Institute of Molecular and Clinical Pathology and Medical Genetics University Hospital Ostrava Ostrava Czechia

The Fingerland Department of Pathology Charles University Faculty of Medicine in Hradec Králové Hradec Králové Czechia

The Fingerland Department of Pathology Charles University University Hospital Hradec Králové Hradec Králové Czechia

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