A Case of Multiple Myeloma Associated with Extramedullary Plasmacytoma of the Gallbladder Manifesting as Acute Cholecystitis.

Multiple myeloma (MM) is a common hematological malignancy that represents 1% of all cancers. MM is distinguished from other plasma cell disorders by prominent bone marrow involvement and systemic organ damage. Extramedullary plasmacytomas of the gall bladder (GB) or biliary ducts, whether solitary or in association with MM, are very rare. We report a case of a 66-year-old female with a history of refractory MM who presented with right upper quadrant abdominal pain. Her laboratory evaluation revealed an abnormal liver panel and lactic acidosis. Abdominal ultrasonography was consistent with acute cholecystitis with no evidence of biliary obstruction or abnormal liver parenchyma. An open cholecystectomy with liver biopsy was performed. The histological evaluation revealed involvement of the GB submucosa and serosa, as well as the liver parenchyma by abnormal plasma cells with lambda light chain restriction. Congo red stain for the GB sample was positive. The patient declined further treatment for MM and was discharged home with comfort measures.

Acute chest syndrome in sickle cell disease.

Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay. The main goal is to prevent and treat acute respiratory failure and, thus, minimize irreversible lung damage. This review focuses on the risk factors, pathogenesis, clinical presentation, and management of ACS.

Strongyloides hyperinfection syndrome and VRE pneumonia.

Immunocompromised patients have high risk of infections from bacteria, viruses, fungi and parasites. One of these infections is those caused by Strongyloides stercoralis Immunocompromised patients are at risk of hyperinfection syndrome which is characterised with more systemic manifestation and a higher risk of morbidity and mortality. This can be complicated by coinfection with enteric organisms, specifically Gram-negative. Enterococci are Gram-positive cocci which are inhabitants of the human gastrointestinal tract. Even though enterococci can cause serious infections in multiple sites, they are a rare cause of pneumonia. We present a case of disseminated strongyloides with vancomycin-resistant enterococcus causing pneumonia. The patient had a complicated course with respiratory failure and septic shock. He died eventually due to his severe infections. After a literature review, we could not find a similar case of coinfection of disseminated strongyloides with vancomycin-resistant enterococcus pneumonia in immune-compromised patients.

Endobronchial hamartoma: a rare disease with more common presentation.

Hamartomas are the most common benign tumours of the lung with an overall incidence of 0.025-0.32%. They are known to be amalgamation of various tissue types, originating from the embryonic mesoderm. Lung hamartomas typically involve lung parenchyma and only infrequently grows as endobronchial tumours. We present a case of an 80-year-old man who presented to the pulmonary clinic for consultation for breathlessness, recurrent pneumonias and an abnormal radiograph finding. CT scan of the chest showed scattered infiltrates and atelectasis in the left upper lobe. He underwent a diagnostic bronchoscopy that showed a lobulated endobronchial lesion obstructing the left upper lobe bronchus. Brush and forceps biopsies were obtained that were consistent with an endobronchial hamartoma. He was referred to the pulmonology department for endobronchial debulking of the lesion following which he improved clinically during postoperative follow-up.

Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease.

Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality with unknown exact aetiology or clear genetic association. It is characterised by a failure of bronchial development and localised glandular overgrowth. Typically, it is diagnosed on prenatal ultrasound, only infrequently in children, and even less commonly in adults. We present a case of a 25-year-old man, with no previous lung diseases who presented with right-sided chest pain, fever and cough suggestive of pulmonary infection. Chest imaging, including CT scan, showed a large focal cystic mass within the right lower lobe along with ground glass opacities suggestive of CPAM. He was started on intravenous antibiotics. Bronchoscopy showed a large amount of pus in the right lung and bronchoalveolar lavage confirmed the microbiological diagnosis of methicillin-resistant Staphylococcus aureus. He improved with antibiotic treatment. He was discharged with 6-week course of antibiotics and follow-up afterward.

Adult Post-Kidney Transplant Familial Atypical Hemolytic Uremic Syndrome Successfully Treated With Eculizumab: A Case Report and Literature Review.

Hemolytic uremic syndrome is the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. When not associated with enteric infection, it is classified as atypical hemolytic uremic syndrome (aHUS) and carries a worse outcome with high mortality rate and up to 50% of the survivors will end up with end-stage renal disease. Renal transplant was restricted to a very small percentage of patients due to high recurrence rate posttransplant that approaches 90%. Our case describes a posttransplant adult patient with familial aHUS whom was successfully treated with eculizumab. We also reviewed all other reported cases of adult posttransplant aHUS, both familial and sporadic, which were treated with eculizumab. In summary, eculizumab might expand the utility of renal transplant for patients with end-stage renal disease due to aHUS.