Refractory Classical Hodgkin Lymphoma Presenting with Atypical Cutaneous Involvement and Diagnosis of ZZ Phenotype Alpha-1 Antitrypsin Deficiency

Abstract

Cutaneous Hodgkin lymphoma is a rare condition. Specific neoplastic involvement can be primary (confined to the skin) or secondary to systemic involvement (metastatic). Cutaneous involvement by HL usually occurs late in the course and is associated with poor prognosis; however in some cases it can exhibit indolent behavior. Skin involvement with nonspecific cutaneous findings may represent a paraneoplastic syndrome. We describe a case of 46-year-old white male patient presented with rash and lymphadenopathy which led to the diagnosis of stage IVE mixed cellularity classical Hodgkin lymphoma with skin involvement. His disease was refractory to multiple lines of chemotherapy including (1) AVD (doxorubicin/bleomycin/dacarbazine), (2) brentuximab, and (3) bendamustine, he later achieved complete remission with (4) GCD (gemcitabine/carboplatin/dexamethasone) salvage regimen. Bleomycin was not given secondary to poor pulmonary function tests. His treatment was complicated after AVD with multiple pneumothoraces which unmasked the diagnosis of ZZ phenotype alpha-1 antitrypsin (ATT) deficiency. Simultaneous existence of Hodgkin lymphoma and ATT is rarely reported.

Figure 1

Pathology microscopy of FNA of the chest wall lesion on presentation: (a) 100x magnification, Papanicolaou stain: Reed-Sternberg cell in a mixed inflammatory background, and (b) 60x magnification, Diff Quik (modified Giemsa) stain: Reed-Sternberg cell in a mixed inflammatory background consisting of neutrophils, histiocytes, and small lymphocytes.

Figure 2

Pathology microscopy of excisional biopsy of a right cervical lymph node (a) showed effacement of the lymph node architecture by a mixed infiltrate of neutrophils, eosinophils, histiocytes, and small lymphocytes, with scattered Reed-Sternberg cells (H&E stain 40x). (b) Immunohistochemical staining for CD30 highlighted the Reed-Sternberg cells (40x).

Figure 3

(a) PET scan at presentation, (b) PET scan done after 6 cycles of AVD, (c) PET scan after 8 cycles of AVD, and (d) PET scan after 2 cycles of brentuximab.

Figure 4

(a) PET scan during brentuximab therapy, (b) PET scan after 2 cycles of bendamustine, (c) PET scan after 2 cycles of GCD, and (d) PET scan at conclusion of treatment with GCD. Pleurodesis inflammatory changes remained on imaging.