Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia

Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. An example is paraneoplastic pemphigus (PNP) which manifests as cutaneous bullae. Bullae may occur anytime during the course of the malignancy i.e. acute phase or remission. Diagnosis involves evaluation of clinical findings, serology and presence of characteristic histological findings. Its pathogenesis is described as development of auto-antibodies against cell junctional and basement membrane proteins. Presence of paraneoplasms has been associated with poorer prognosis and increased mortality in hematological malignancies including CLL. Currently, there are established indications for the treatment of CLL; however, presence of paraneoplasms as an indication for treatment is unclear. Patients with paraneoplasms who underwent expeditious treatment have exhibited better clinical outcomes. Herein we describe a case of a CLL patient in remission presenting with PNP and its response to treatment

Cholangiocarcinoma Presenting as Humoral Hypercalcemia of Malignancy: A Case Report and Literature Review

Humoral hypercalcemia of malignancy (HHM) is most commonly encountered in squamous cell carcinoma (SCC) of different organs, and It is characterized by elevated parathyroid hormone-related peptide (PTHrP) levels. It may be seen as a manifestation of cholangiocarcinoma (CCC) at presentation and later in the course of the disease. However, HHM due to intrahepatic cholangiocarcinoma is a rare association and is associated with a poor prognosis. We herein report a case of hypercalcemia presenting as the first manifestation of an underlying rare variant of intrahepatic cholangiocarcinoma. Our patient is a 57-year-old male who presented to the emergency room with severe symptoms of constipation and polyuria and was admitted to the hospital for symptomatic hypercalcemia. He was found to have a hypermetabolic 15 cm liver mass, abdominal lymph nodes on imaging, which was subsequently diagnosed as adenosquamous cholangiocarcinoma by liver biopsy. This necessitated an urgent inpatient treatment with gemcitabine/cisplatin combination chemotherapy to control the aggressive nature of the malignancy. However, he deteriorated and expired after three months of his diagnosis. Adenosquamous cholangiocarcinoma is a very rare variant of a liver tumor. It develops due to squamous metaplasia of an underlying cholangiocarcinoma and usually has aggressive clinicopathological features. HMM is a life-threatening, yet unrecognized, phenomenon of cholangiocarcinoma, which represents a poor prognostic marker. Prompt recognition of this complication is important for preventing serious complications associated with hypercalcemia and to improve the quality of life of these patients