A Rare Case of Spontaneous Tumor Lysis Syndrome in Hodgkin Lymphoma.

Tumor lysis syndrome (TLS) is an oncological emergency characterized by biochemical abnormalities such as metabolic acidosis, hyperkalemia, hyperphosphatemia, and hypocalcemia. The clinical outcome is directly related to the biochemical abnormalities. TLS can occur in any malignancy, but it is highly associated with rapidly proliferating tumors. Although the syndrome is commonly associated with hematological malignancies, particularly with leukemia and non-Hodgkin's lymphoma, it is rarely seen in patients with Hodgkin's lymphoma. In our case, a 7-year-old girl presented with intermittent fever, non-productive cough, fatigue, and night sweats for four months. On examination, she had an enlarged cervical lymph node of 5 cm in size on the left side accompanied by palpable supraclavicular lymphadenopathy. Past medical history was significant for the relapsing and remitting course of nephrotic syndrome diagnosed two years before presentation. The patient underwent a left-sided cervical node excisional biopsy, which confirmed classical Hodgkin's lymphoma of mixed cellularity type. Her baseline chest x-ray revealed a bulky anterior mediastinal mass. To stage the tumor, a bone marrow biopsy, CT, and positron emission tomography (PET) scan were done. Although the bone marrow biopsy report showed a normal pattern of trilineage hematopoiesis, the CT and PET scan results led to its classification under stage 4. During her stay in the hospital for further work-up and treatment, her condition suddenly deteriorated. There were biochemical derangements on lab reports that confirmed the Spontaneous Tumor Lysis Syndrome (STLS). She recovered completely due to immediate stabilization and correction of electrolyte abnormalities. STLS is a life-threatening condition that is rarely seen in patients with Hodgkin's lymphoma. The treating physicians should be vigilant about this possible sequela of Hodgkin's lymphoma and be aware of its different presentations.

Pilomatrixoma of the Left Scapular Region in a Young Male: A Rare Case Report.

Pilomatrixoma is a benign and rare neoplasm derived from the cortex of the hair follicle. The head and neck region's involvement is relatively common and occurs in the upper extremity, trunk, and lower extremity in decreasing tendency. It is usually encountered in younger age groups, usually in children and adolescents. Management of the pilomatrixoma involves surgical excision of the mass. A 15-year-old male presented with a history of an isolated left scapular mass. The lesion had appeared two weeks before the presentation. His parents brought him to the emergency department due to continued bleeding from the mass. He denied any pain, fever, night sweats, weight loss, chills, appetite changes, and numbness to the area. Physical examination revealed a bleeding non-tender mass measuring about 2.5 x 3 cm, with the fluctuant inferior surface. Baseline investigations were normal. Cyst, abscess, and neoplasm were the initial differentials. Ultrasound findings were suggestive of neoplasm, and contrast-enhanced MRI was highly suspicious for progressive neoplasm. The surgical team was consulted, and surgical excision of the mass was carried out. Biopsy results confirmed pilomatrixoma. In conclusion, the pre-surgical diagnosis of pilomatrixoma is difficult. Hence, this case report's main objective is to raise awareness among clinicians about this rare diagnosis and its clinical features. Furthermore, our case highlights the need to consider pilomatrixoma in the differentials of the head, neck, and back masses in children and adolescents.

Lamin A/C Cardiomyopathy with E203K Pathogenic Mutation.

Lamin A/C (LMNA) cardiomyopathy is an adult-onset, autosomal dominant, rapidly progressive cardiomyopathy which belongs to a spectrum of familial idiopathic cardiomyopathies. It is the most common type of familial dilated cardiomyopathy that is associated with conduction defects. A 76-year-old African American female with second-degree atrioventricular (AV) block presented for evaluation of persistent fatigue. Her family history was significant for sudden deaths of her son and brother at the age of 6 and 48 years, respectively, and AV block in her sister with a pacemaker implant at the age of 64 years. Physical examination was within normal limits. Electrocardiogram showed a Mobitz type II, second-degree AV block. Mild dilated cardiomyopathy was present on echocardiogram. Stress echocardiography had to be stopped due to premature ventricular contractions. Cardiac catheterization, coronary angiography, and cardiac MRI revealed no significant etiology for rhythm disturbance. Holter monitoring revealed intermittent bradycardia with a heart rate falling as low as 28 beats per minute, which led to the decision of dual-chamber pacemaker implantation. RhythmNext genetic testing (Ambry Genetics, Aliso Viejo, CA) was done due to the significant family history of sudden death; it revealed a heterozygous E203K pathologic mutation in the LMNA gene. Sudden death is the most common mode of death in LMNA cardiomyopathy; hence, the implantation of intracardiac cardioverter-defibrillator for primary prophylaxis was discussed with the patient. Clinicians should suspect LMNA cardiomyopathy in patients with rhythm disorders and family history of sudden death, which can help to identify individuals at risk and prevent sudden death by appropriate interventions.