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

[hodgkin lymphoma, classical]

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 .