Tuberc Respir Dis.  2016 Oct;79(4):302-306. 10.4046/trd.2016.79.4.302.

Pulmonary Leukocytoclastic Vasculitis as an Initial Presentation of Myelodysplastic Syndrome

• Lee SH
• Kim JH
• Park S
• Won CY
• Lee JH
• Yi SY
• Park HK
• Chang SH
• Jung H
• Lee SS
• Koo HK

• KMID: 2365323
• DOI: http://doi.org/10.4046/trd.2016.79.4.302

Abstract

Systemic vasculitis involving the lung is a rare manifestation of myelodysplastic syndrome (MDS), and secondary vasculitis is considered to have poor prognosis. A 44-year-old man presented with fever and dyspnea of 1 month duration. A chest radiograph revealed bilateral multiple wedge shaped consolidations. In addition, the results of a percutaneous needle biopsy for non-resolving pneumonia were compatible with pulmonary vasculitis. Bone marrow biopsy was performed due to the persistence of unexplained anemia and the patient was diagnosed with MDS. We reported a case of secondary vasculitis presenting as non-resolving pneumonia, later diagnosed as paraneoplastic syndrome of undiagnosed MDS. The cytopenia and vasculitis improved after a short course of glucocorticoid treatment, and there was no recurrence despite the progression of underlying MDS.

Keyword

Myelodysplastic Syndromes; Vasculitis, Leukocytoclastic, Cutaneous; Paraneoplastic Syndromes; Pneumonia

MeSH Terms

Adult
Anemia
Biopsy
Biopsy, Needle
Bone Marrow
Dyspnea
Fever
Humans
Lung
Myelodysplastic Syndromes*
Paraneoplastic Syndromes
Pneumonia
Prognosis
Radiography, Thoracic
Recurrence
Systemic Vasculitis
Vasculitis*
Vasculitis, Leukocytoclastic, Cutaneous

Figure

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  Figure 1 Initial chest radiograph (A) and chest computed tomography scan (B).

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  Figure 2 Cutaneous lesions of bilateral lower legs were later revealed as cutaneous leukocytoclastic vasculitis.

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  Figure 3 (A) Heavy, perivascular and interstitial neutrophilic infiltration (arrowhead) are present (H&E stain, ×40). (B) Fibrinoid necrosis of the small blood vessels with fibrin extravasation and leukocytosis forming nuclear dust (arrowhead) are visible on skin biopsy (H&E stain, ×200).

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  Figure 4 Neutrophilic infiltration and fibrinoid necrosis of the blood vessel wall are shown, and alveolar space is filled with fibrinoid exudate on lung biopsy (H&E stain, ×200).

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  Figure 5 Chest radiographs post-steroid therapy at 3 days (A) and 1 year (B).

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