PATIENTS CONCERNS: The patient was diagnosed with HCC, presented 5 months later with right lower tooth pain, swelling over the right mandible area and right shoulder pain.
DIAGNOSES: Histopathological examination of mandible showed findings suggestive of metastatic HCC. Magnetic resonance imaging (MRI) of the right shoulder revealed findings of irregular enhancing lesion at the right coracoid process causing erosion of the coracoid process.
INTERVENTIONS: Patient was subsequently referred for palliative medicine care.
OUTCOMES: He received adequate analgesia.
LESSONS: Oral cavity and scapula metastases from HCC are very rare. Most oral metastases are associated with lung metastases, and they possibly occur by hematogenous route. In our case, the possible pathway of metastasis is an anastomotic network of paravertebral veins that bypasses the pulmonary, inferior caval, and portal venous circulations.
PATIENTS CONCERNS: The patient presented with 5 months' history of generalized skin itchiness, night sweat and loss of weight. The skin manifestations started over the foot and hand area. However, he started to developed tense blisters over the face, trunk and limbs 3 days prior to this admission.
DIAGNOSES: The skin biopsy report showed subepidermal bullae, in which the immunofluorescence findings in keeping with bullous pemphigoid. The peripheral blood immunophenotyping was suggestive of mantle cell lymphoma. Hence, a diagnosis of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma was made.
INTERVENTIONS: The patient was initiated with a cytoreduction chemotherapy.
OUTCOMES: Unfortunately, patient's condition deteriorated further due to neutropenic sepsis and he succumbed after 2 weeks of intensive care.
LESSONS: Bullous pemphigoid associated with mantle cell lymphoma are very rare. The presentation of bullous pemphigoid led to the detection of mantle cell lymphoma. Early diagnosis and appropriate treatment is crucial in managing this aggressive type of the disease. Both, bullous pemphigoid and mantle cell lymphoma had a parallel clinical course which suggests a paraneoplastic phenomenon in this reported case.
METHODS: Subjects were recruited from lipid and cardiac specialist hospitals. FH was clinically diagnosed using the Dutch Lipid Clinic Network Criteria. Patients' medical history was recorded using a standardized questionnaire. LLM prescription history and baseline LDL-C were acquired from the hospitals' database. Blood samples were acquired for the latest lipid profile assay.
RESULTS: A total of 206 patients with FH were recruited. Almost all of them were on LLMs (97.6%). Only 2.9% and 7.8% of the patients achieved the target LDL-C of <1.4 and <1.8 mmol/L, respectively. The majority of patients who achieved the target LDL-C were prescribed with statin-ezetimibe combination medications and high-intensity or moderate-intensity statins. All patients who were prescribed with ezetimibe monotherapy did not achieve the target LDL-C.
CONCLUSION: The majority of Malaysian patients with FH received LLMs, but only a small fraction achieved the therapeutic target LDL-C level. Further investigation has to be conducted to identify the cause of the suboptimal treatment target attainment, be it the factors of patients or the prescription practice.