Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease.

Sickle cell disease is one of the most common inherited hemoglobinopathies diagnosed in the United States. Patients often present with severe anemia, pain crises, infections, and vaso-occlusive phenomena. Complications of these disorders can lead to significant debilitating morbidity and mortality. Fat embolism syndrome (FES) is a rare and devastating complication of sickle cell disease. It usually presents with a rapidly deteriorating clinical course, and the prognosis is dismal. We report a case of FES in a 19-year-old African American male with a history of sickle cell disease who presented with tonic-clonic seizures and was found to have multi-organ failure. FES was diagnosed 20 days from a presentation based on blood cytopenias and magnetic resonance imaging findings that were obscured at the initial presentation. We describe in this report, the patient's course from presentation until diagnosis and resolution. Our case is peculiar as the patient had a very good outcome without the need for red blood cell (RBC) exchange; instead, supportive treatment and simple RBC transfusions were enough to change the clinical course of this almost fatal syndrome.

Venous thromboembolism prophylaxis in hospitalized sickle cell disease and sickle cell trait patients.

INTRODUCTION: Sickle trait (Hb SA) or sickle disease (Hb SS) carries increased risk of venous thromboembolism (VTE). Hb SS patients are young and lack common comorbid conditions that qualify them for VTE prophylaxis (VTEP). METHODS: Retrospective, multicenter analysis of Hb SS/Hb SA adult patients between January 2013 and December 2018. RESULTS: There were 803 Hb SA (525 patients) and 1020 Hb SS admissions (262 patients). VTEP use was similar between Hb SA and controls (42% vs. 46%; p-value = .06) and Hb SS and controls (45% vs. 42%; p-value = .13). Hb SS/Hb SA patients more frequently received more than half of prescribed doses of VTEP. In multivariate analysis, increasing age and longer hospitalizations were positive predictors. Odds of VTEP use varied with treatment site for Hb SS patients, whereas comorbid conditions, admission hemoglobin and platelet count were not predictive. By contrast, in Hb SA patients, comorbid conditions, higher admission hemoglobin, and higher admission platelet counts raised the odds of VTEP being offered. CONCLUSIONS: VTEP is underused in Hb SS/Hb SA patients. There may be a trend toward offering more VTEP in Hb SS disease, but not in Hb SA patients, where VTEP prescribing is driven by comorbid conditions rather than genotype. Patient compliance does not appear to play a major role, but intercenter variability suggests provider education may improve VTEP use.

NUT midline lung cancer: a rare case report with literature review.

Nuclear carcinoma of the testis (NUT) midline carcinoma are rare, poorly differentiated tumors resulting from t(15; 19) rearrangement, clinically characterized by aggressive and rapid progression to death. No optimal treatment regimen has been established for this rare malignancy. Surgery, chemotherapy, and radiation have been used for treatment alone or in combination, depending on location and staging of the disease, and may confer short periods of remission; however, re-emergence of the disease inevitably occurs. Targeted therapies such as bromodomain and extra-terminal domain protein (BET) inhibitors are currently in early phases of clinical trials. Here we describe a 49-year-old-male with no comorbidities who presented with acute worsening of chronic cough, new onset hemoptysis and left sided chest pain for 2 weeks. Workup revealed stage IIIB NUT midline carcinoma (NMC) of the lung with next-generation sequencing confirming the presence of a NUTM1-BRD4 fusion. The tumor was unresectable, and he began concurrent chemoradiation with weekly carboplatin and paclitaxel for 5 weeks. The follow-up CT scan showed partial response, so maintenance was continued with durvalumab. Two months later, he presented with metastasis to the posterior muscle compartment of the left arm, which was treated with local radiotherapy. Four months later he developed progression of lung disease with multiple pulmonary nodules. Durvalumab was discontinued and he was prescribed the BET inhibitor molibresib, 120 mg daily. After nearly 3 months of treatment with molibresib, he presented with brain metastasis for which he had a craniotomy with tumor resection and gamma knife radiation to solitary metastatic lesions. He was then prescribed chemo-immunotherapy with carboplatin plus pemetrexed and pembrolizumab. After two cycles of treatment his disease progressed, and he succumbed to it. Total survival was 18 months. In conclusion, NUT midline lung carcinoma is a rare but aggressive malignancy and patients have limited treatment options especially in advanced stages. Few targeted therapies have shown promising results in early clinical trials but more treatment options are awaited.

Chalazion in a patient with multiple myeloma treated with bortezomib.

We present a case of a woman in her early 60s with multiple myeloma who, while undergoing treatment with cyclophosphamide, bortezomib and dexamethasone (CyBorD), noticed a whitish nodular swelling on the eyelid. This occurred after one cycle of CyBorD and on subsequent treatment, it also involved the contralateral eyelid. The lesions were initially managed with conservative measures by applying warm compresses, but the lesions progressively increased in size. CyBorD was discontinued and topical antibiotics and anti-inflammatories were initiated, resulting in a decrease in size of the lesions. On resolution of symptoms, she was rechallenged with CyBorD, and symptoms did not recur. The temporal relationship between bortezomib and the development of chalazion is based on connection and no association has been proven.

Leucocytoclastic Vasculitis, Cryoglobulinemia, or Plasma Cell Leukemia: A Diagnostic Conundrum.

Plasma cell leukemia is rare and could be life-threatening. Even rarer and equally life-threatening is cryoglobulinemia. Both of them occurring together paints a grim clinical picture. We present the case of a 63-year-old male with plasma cell leukemia complicated by cryoglobulinemia with skin lesions. The report briefly reviews the clinical and diagnostic characteristics of plasma cell leukemia and well as available treatment options. It also highlights the need to consider non-chemotherapy-based regimens and clinical trials in the care of plasma cell leukemia patients.

Paradigm shift in the management of metastatic nonsmall cell lung cancer.

BACKGROUND: Lung cancer is one of the leading causes of cancer mortality in the United States. The use of precision medicine in the past 10 years has significantly changed the therapeutic landscape of lung cancer. Management of advanced nonsmall cell lung cancer (NSCLC) has transitioned from a chemotherapeutic approach to targeted treatments and immunotherapeutic agents. Several tyrosine kinase inhibitors (TKIs) have been approved for patients with targeted mutations and patients who do not have driver mutations; immunotherapy has been recently approved as frontline therapy, which has resulted in marked improvement in overall survival and added a new tool in our armamentarium. AIMS: The purpose of this review is to highlight recent advancements in diagnostic approach and management strategies in patients with metastatic NSCLC. MATERIALS AND METHODS: A literature search was conducted on Medline (via PubMed) and National Comprehensive Cancer Network Guidelines using the keywords "precision diagnosis," "advanced non-small cell lung cancer," "target therapies," and "immunotherapy." CONCLUSION: The use of next-generation sequencing has significantly changed our understanding of molecular oncogenic mechanisms of lung cancer. These advancements have created a paradigm shift in the treatment strategies of metastatic lung cancer from primarily chemotherapeutic approach to increasing use of targeted therapies and immune checkpoint inhibitors (ICI) leading to better survival rates and lesser toxicity.

Epstein-Barr virus induced haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis (HLH) is a rare condition of uncontrolled immune activation as a result of an inherited genetic defect or in response to malignancy, autoimmune disease, rheumatological disease, AIDS infection or post-transplant immunosuppression. Described here is the case of a 19-year-old Caucasian man who presented with complaints of worsening fever, new-onset jaundice and lethargy after failing treatment for suspected infectious mononucleosis. Physical examination was significant for fever and splenomegaly while laboratory results revealed transaminitis, cytopaenia, indirect hyperbilirubinaemia and elevated ferritin, raising the likelihood of both autoimmune haemolytic anaemia and HLH. He tested positive for Epstein-Barr virus (EBV), and bone marrow biopsy revealed hypercellular marrow with haemophagocytosis and no evidence of malignancy. High dose steroids were initiated with significant improvement in haemoglobin, resulting in a final diagnosis of HLH secondary to acute EBV infection. The patient was discharged on continued high-dose prednisone with planned taper and consideration of outpatient rituximab therapy for 4 weeks. High clinical suspicion and prompt evaluation were critical to early treatment and decreased morbidity.

Coronavirus Disease 2019 and Cold Agglutinin Syndrome: An Interesting Case.

The coronavirus disease 2019 (COVID-19) pandemic has caused significant morbidity and mortality worldwide. While patients with COVID-19 most frequently present with pneumonia, respiratory failure and acute respiratory distress syndrome, increasing cases of immune-mediated disorders such as autoimmune thrombocytopenia, haemolytic anaemia and antiphospholipid syndrome have been reported. In this article we describe a rare case of cold agglutinin syndrome (CAS) in a patient with COVID-19. The patient was a 77-year-old man with a history of glucose-6-phosphate dehydrogenase (G6PD) deficiency who presented with COVID-19 infection and acute respiratory failure. Initially he was started on intravenous steroids, antibiotics and hydroxychloroquine. Laboratory analysis revealed haemolytic anaemia with a positive direct anti-globulin test (DAT) and high titres of cold agglutinins. Hydroxychloroquine was stopped due to suspicion of haemolysis due to G6PD deficiency but the haemolysis persisted. Unfortunately, the respiratory failure progressed and the patient died. In summary, this article describes a rare case of CAS associated with COVID-19. CAS is a heterogenous group of cold autoimmune haemolytic anaemias occurring secondary to infections or malignancies. No definite treatment for CAS in COVID-19 patients has been approved so far. LEARNING POINTS: Autoimmune haemolytic anaemia has been reported in COVID-19 patients.Cold agglutinin syndrome (CAS) can occur in patients with COVID-19.Efforts to determine the optimal management of CAS in COVID-19 patients must continue.

Inferior Vena Cava Filter Retrieval Trends: A Single-Center Experience.

Recognition of the adverse events of inferior vena cava filters (VCFs) has prompted the Food and Drug Administration (FDA) to issue safety warnings (2010 and 2014), advocating for removal, once the risk of pulmonary embolism has abated. Despite an initial increase in retrieval rates, these remain low (25-30% at 1 year in 2014). We retrospectively investigated retrieval trends in adults with VCFs placed between 2015 and 2018 at a single institution. The rate of retrievable VCF removal accounting for the competing risk of death was the main outcome. There were 494 VCFs placed (305 retrievable). The cumulative incidence of retrieval remained low (21% at 1 year), even after the second FDA warning (2014). Patients who resumed anticoagulation (AC) at any time were more likely to have retrieval (hazard ratio [HR] = 3.6, p < 0.01) and had higher retrieval rates at every time point (31.4 vs. 7.6% at 1 year). Advanced age (HR = 0.98 per year, p = 0.004), stroke (HR = 0.28, p = 0.028), and active malignancy (HR = 0.42, p = 0.006) predicted nonretrieval. Device-related complications were infrequent (<1%) but thrombotic complications occurred early and were more common for nonretrieved VCFs (17 vs. 12%, p = 0.29). Revision of guidelines to recommend active surveillance for the ability to tolerate AC in the immediate postimplantation period may improve retrieval rates.

Extranodal blastoid/pleomorphic variant of mantle cell lymphoma involving the testis and skin.

We report a case of extranodal mantle cell lymphoma of the testis in a 72-year-old Caucasian man who presented to his physician's office with rapidly enlarging left testicular mass and skin lesions which were subsequently biopsy-proven to be mantle cell lymphoma. We discuss the clinicopathological features and current management in relapsed and refractory settings of this rare presentation of mantle cell lymphoma.

Diffuse large B-cell lymphoma of the vagina in pregnancy.

A 28-year-old primigravida was evaluated for complaints of difficulty urinating and pelvic pain of 6-weeks duration. She denied fever, night sweats, weight loss or fatigue. Pelvic ultrasonography revealed a single fetal pole with cardiac activity and a 7 cm mass in the anterior vagina which encased the urethra. The diagnosis of diffuse large B-cell lymphoma germinal centre type was made on analysis of biopsied pelvic mass. Whole body MRI revealed the disease was limited to the vagina. The patient received six cycles of Rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone with significant improvement in her symptoms. Serial ultrasounds over the subsequent months showed appropriate development of the fetus. Whole body MRI after treatment showed decreased size and decreased signal of the primary pelvic mass compatible with favourable treatment response. Challenges in the management of this rare presentation of lymphoma are discussed.

When leucocytosis is not leukaemia.

A female aged 84 years with a history of Clostridium difficile-associated diarrhoea presented from an extended care facility with altered mental status and respiratory distress. She was haemodynamically unstable and initial laboratory results revealed hyperleucocytosis (110.3×109/L). The presence of immature myeloid precursors, thrombocytopenia and respiratory distress, raised concern for an acute leukaemic process requiring emergent leucapheresis. However, on evaluation of the peripheral smear, prominent left shift and toxic granulation were noted, along with absence of blast cells. Considering her history of C. difficile infection, a CT scan of the abdomen and pelvis was obtained, which was suggestive of toxic megacolon. She was taken to the operating room for emergent colectomy. The pathology specimen showed pseudomembrane formation consistent with fulminant C. difficile infection. She was treated with oral vancomycin and intravenous metronidazole, followed by clinical improvement and resolution of leucocytosis and thrombocytopenia.

Hepatotoxicity due to Clindamycin in Combination with Acetaminophen in a 62-Year-Old African American Female: A Case Report and Review of the Literature.

Clindamycin is a bacteriostatic lincosamide antibiotic with a broad spectrum. Side effects include nausea, vomiting, diarrhea, and metallic taste; however, hepatotoxicity is rare. The incidence is unknown. It is characterized by increases in aspartate and alanine transaminases. There may be no symptoms and the treatment is to stop the administration of clindamycin. We have described a 62-year-old African American female medicated with acetaminophen and clindamycin who had initially presented to the dental clinic for the evaluation of gum pain following tooth extraction. She had significantly increased levels of liver transaminases, which trended downwards on quitting the medication.

Unusual Spread of Renal Cell Carcinoma to the Clivus with Cranial Nerve Deficit.

Renal cell carcinoma (RCC) has unusual presentation affecting elderly males with a smoking history. The incidence of RCC varies while the incidence of spread of RCC to the clivus is rare. The typicality of RCC presentation includes hematuria, flank pain, and a palpable flank mass; however, RCC can also present with clival metastasis. The unique path of the abducens nerve in the clivus makes it susceptible to damage in metastasis. We report a case of a 54-year-old African American female that was evaluated for back pain, weakness, numbness, and tingling of bilateral lower extremities and subsequently disconjugate gaze and diplopia. Brain MRI confirmed metastasis to the clivus. She was started on radiotherapy and was planned for chemotherapy and transfer to a nursing home. When a patient presents with sudden unusual cranial nerve pathology, the possibility of metastatic RCC should be sought.

Pancytopenia in a 70-year-old african-american male: an unusual presentation of a rare disease.

Hairy cell leukemia is a rare lymphoid neoplasm arising from mature B-lymphocytes. Clinically, the disease presents with splenomegaly and abdominal discomfort, frequent infections, fatigue and bleeding because of related cytopenias. Bone marrow biopsy is essential for diagnosis. Below we describe a case of a 70-year-old African-American male who presented to our hematology clinic complaining of fatigue. Clinical exam and computed tomography imaging did not reveal splenic enlargement. Blood work-up revealed pancytopenia and bone marrow was diagnostic for hairy cell leukemia.The patient was started on cladribine, with gradual improvement of his symptoms and blood count abnormalities. Therefore, it is essential to keep hairy cell leukemia in the differential of pancytopenia even in the absence of a splenomegaly.

Malignant mesothelioma after household exposure to asbestos.

Malignant mesothelioma (MM) is an aggressive cancer that has been closely linked to asbestos exposure. Initially recognized as an occupational cancer in male workers, MM was later found to occur in their family members as well. We report the case of an 89-year-old female who presented with abdominal distention, pain, and findings consistent with malignant ascites. Family history was significant for fatal mesothelioma in her husband of 40 years, who was a worker at a tile factory. The diagnosis of MM was confirmed on pathologic examination of the omental core biopsy.