An improved index for diagnosis and mortality prediction in malignancy-associated hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome that may complicate hematologic malignancies (HMs). The appropriateness of current criteria for diagnosing HLH in the context of HMs is unknown because they were developed for children with familial HLH (HLH-2004) or derived from adult patient cohorts in which HMs were underrepresented (HScore). Moreover, many features of these criteria may directly reflect the underlying HM rather than an abnormal inflammatory state. To improve and potentially simplify HLH diagnosis in patients with HMs, we studied an international cohort of 225 adult patients with various HMs both with and without HLH and for whom HLH-2004 criteria were available. Classification and regression tree and receiver-operating curve analyses were used to identify the most useful diagnostic and prognostic parameters and to optimize laboratory cutoff values. Combined elevation of soluble CD25 (>3900 U/mL) and ferritin (>1000 ng/mL) best identified HLH-2004-defining features (sensitivity, 84%; specificity, 81%). Moreover, this combination, which we term the optimized HLH inflammatory (OHI) index, was highly predictive of mortality (hazard ratio, 4.3; 95% confidence interval, 3.0-6.2) across diverse HMs. Furthermore, the OHI index identified a large group of patients with high mortality risk who were not defined as having HLH according to HLH-2004/HScore. Finally, the OHI index shows diagnostic and prognostic value when used for routine surveillance of patients with newly diagnosed HMs as well as those with clinically suspected HLH. Thus, we conclude that the OHI index identifies patients with HM and an inflammatory state associated with a high mortality risk and warrants further prospective validation.

JAK 2-Positive Diseases and Spontaneous Coronary Artery Dissection: Case Series.

Introduction: Chronic myeloproliferative neoplasms (MPNs) such as polycythaemia vera (PV) and essential thrombocytosis are associated with atherosclerosis and cardiovascular events, especially if JAK2 mutation is there. In rare cases, also spontaneous coronary artery dissection (SCAD) can be seen. Case Presentation: This case series describes two case reports of MPNs in which SCAD was found in both of them in addition to standard atherosclerosis. First one was about a 42-year-old man who was admitted for acute myocardial infarction (MI) and was later found to have PV and SCAD. The second one was about a 52-year-old man who was also admitted for acute MI and was later found to have SCAD and essential thrombocythemia. JAK2V617F missense mutation was found in both cases. Conclusion: MPNs with JAK2 mutations are associated with SCAD in addition to usual atherosclerosis. This association needs further research.

Characteristics and outcomes of children, adolescent, and young adult patients with myelodysplastic neoplasms: A single-center retrospective analysis.

Myelodysplastic syndrome, or myelodysplastic neoplasms, are a rare finding in pediatric, adolescent, and young adult (AYA) patients. More literature is needed to highlight trends of survival or treatment resistance in subpopulations to improve treatment. Here we report a single center retrospective analysis of pediatric and AYA patients from 2000 to 2022 including molecular and cytogenetic data. Using the IPSS-R and IPSS-M, which have been reported exclusively in adults, and excluding patients with bone marrow failure syndromes, we analyzed 119 pediatric and AYA patients with myelodysplastic neoplasms. Therapy-related myelodysplastic neoplasms were present in 36 % of patients, and 31 % of patients developed acute myeloid leukemia. The 5-year overall survival (OS) rate for the entire cohort was 45 %. Contrary to young adults and older adults, mutations were not common in pediatrics. Those who underwent stem cell transplant (SCT)(at any time) had significantly longer median OS. Although SCT at any time improved OS in the de novo myelodysplastic neoplasm group, the choice of the initial treatment with intensive chemotherapy, hypomethylating agents, or SCT did not significantly alter OS. Median OS was shorter in the pediatric group (<18 years old) and longer for those with isolated deletion of 5q or TET2 mutation, but these were not significant findings. Median OS was significantly shorter in those with monosomy 7 or 7q deletion and those with therapy-related myelodysplastic neoplasms. These findings build on previously reported findings and encourage the use of SCT along with molecular and cytogenetic analysis.

Case Report: Tuberculosis-Induced Autoimmune Hemolytic Anemia.

Tuberculosis (TB) is an infectious disease that affects different organs, causing multiple complications, including hematological sequelae. One of the most common TB-hematological complications is anemia of chronic disease. Very rarely autoimmune hemolytic anemia (AIHA) has been reported as an uncommon manifestation of TB. In this case, we present a female who presented with refractory AIHA, which was attributed to disseminated TB. The patient responded well to steroids, anti-TB medications, and rituximab.

Central catheter-related Gordonia bronchialis bacteremia in an immunocompromised patient: A case report, and literature review.

Gordonia is a rarely reported organism causing central line-associated bloodstream infection (CLABSI). This article reports an acute myeloid leukemia (AML) case in which the patient developed febrile neutropenia and was later found to have Gordonia bronchialis (G. bronchialis) CLABSI. The patient received a two-week ceftriaxone regimen, based on susceptibility. The microbiologic diagnosis of this organism is considered challenging due to its resemblance with other organisms; however, more sophisticated methods of diagnosis (such as gene sequencing) can aid in differentiation.

Hyperosmolar Hyperglycaemic State and Diabetic Ketoacidosis in Nivolumab-Induced Insulin-Dependent Diabetes Mellitus.

Nivolumab is a monoclonal antibody directed against programmed cell death-1 receptor. It has an increasing application in the treatment of various advanced metastatic cancers. The incidence of autoimmune side effects associated with such agents is expected to increase. New-onset autoimmune diabetes mellitus associated with immune checkpoint inhibitor treatment is rare, occurring in less than 1% of patients. Nivolumab-induced diabetes often presents as diabetic ketoacidosis, which could be life-threatening if not recognized and treated promptly. We present the case of a patient who developed severe diabetic ketoacidosis concomitant with hyperosmolar hyperglycaemic state (HHS) after receiving nivolumab for metastatic testicular lymphoma. Pre-nivolumab blood glucose levels were normal, apart from transient hyperglycaemia related to steroids as part of the chemotherapy protocol. The diagnosis was confirmed with extremely low C-peptide in the clinic. LEARNING POINTS: Checkpoint inhibitor-associated diabetes can present abruptly with life-threatening complications.Most patients require multiple daily injections of insulin upon discharge.Cessation of checkpoint inhibitor therapy does not revert diabetes.

Imatinib-Induced Tremor in a Patient with Chronic Myeloid Leukemia in Chronic Phase.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by three phases: chronic, accelerated, and blast phase. However; first- and second-generation tyrosine kinase inhibitors are used for the treatment of CML with common and uncommon adverse events. Here, we report a 24-year-old male with CML in chronic phase started on imatinib as upfront medication who developed tremor and recovered spontenously after 3 years.

Acute Bacterial Meningitis in Qatar: A Hospital-Based Study from 2009 to 2013.

BACKGROUND AND OBJECTIVES: Bacterial meningitis is a common medical condition in Qatar. The aim of this study was to describe the clinical characteristics of bacterial meningitis, the frequency of each pathogen, and its sensitivity to antibiotics and risk factors for death. PATIENTS AND METHODS: This retrospective study was conducted at Hamad General Hospital between January 1, 2009, and December 31, 2013. RESULTS: We identified 117 episodes of acute bacterial meningitis in 110 patients. Their mean age was 26.4 ± 22.3 years (range: 2-74) and 81 (69.2%) of them were male patients. Fifty-nine episodes (50.4%) were community-acquired infection and fever was the most frequent symptom (94%), whereas neurosurgery is the most common underlying condition. Coagulase-negative staphylococci were the most common causative agent, of which 95% were oxacillin-resistant, while 63.3% of Acinetobacter spp. showed resistance to meropenem. The in-hospital mortality was 14 (12%). Only the presence of underlying diseases, hypotension, and inappropriate treatment were found to be independent predictors of mortality. CONCLUSION: Acute bacterial meningitis predominantly affected adults and coagulase-negative staphylococci species were the common causative agent in Qatar with majority of infections occurring nosocomially. More than 90% of all implicated coagulase-negative staphylococci strains were oxacillin-resistant.

Characteristics of patients with definite septic arthritis at Hamad General Hospital, Qatar: a hospital-based study from 2006 to 2011.

The aim of this retrospective study was to determine the epidemiological and clinical characteristics, coexisting conditions, causative organisms, and outcomes of all adult patients 15 years of age or older who had definite septic arthritis at Hamad General Hospital, Qatar, from 2006 to 2011. During this period, 56 patients were diagnosed with septic arthritis (mean age ± SD, 49.0 ± 16.6 years). In 53 of 56 (94.6%) patients, arthritis was diagnosed in a single joint, while polyarthritis was diagnosed in 3 of 56 (5.4%) patients; the most commonly involved joint was the knee (40 of 59 joints, 67.7%). The most frequent coexisting condition was diabetes mellitus (24 of 56 patients, 42.8%). Joint pain and restriction of movement were reported by all patients. Gram-positive bacteria accounted for 36 of all 57 (63.0%) isolated microorganisms, and Staphylococcus aureus was the most common pathogen (20 of 57 microorganisms, 35.0%). Three cases of tuberculous arthritis were seen. The most favored antibiotic combinations were cloxacillin/ciprofloxacin, cefazolin/ciprofloxacin, and vancomycin/ciprofloxacin. Repeated needle aspiration, open joint drainage, and arthroscopic techniques were performed in 18 (32.1%), 22 (39.3%), and 11 (19.6%) of the 56 patients, respectively. The 30-day mortality was 3.6%, and the remaining patients showed clinical improvement upon discharge. In conclusion, there was no specific sign or symptom for diagnosing septic arthritis. Isolation of bacteria from the synovial fluid confirmed the diagnosis, and S. aureus and streptococci were the most common pathogens isolated. Prompt treatment with appropriate antibiotics and synovial drainage are mandatory to improve the outcome.

Epidemiological features of Clostridium difficile infection among inpatients at Hamad General Hospital in the state of Qatar, 2006-2009.

The aim of this retrospective study was to evaluate the epidemiology, clinical course and outcome of Clostridium difficile infection among inpatients at Hamad General Hospital in Qatar, from 2006 to 2009. During this period, 123 patients were diagnosed with C. difficile infection and the overall incidence was 1.6/10,000 patient days. The mean age (±SD) of patients was 50.9 ± 21.2 years. The most frequent underlying disease was hypertension 51/123 (41.5%) and 133 prescriptions of antimicrobials were ordered for 105/123 (86.1%) patients prior to C. difficile infection with piperacillin-tazobactam being the most frequently prescribed antimicrobial 39/131 (29.7%). Nosocomial infection was found in 101/123 (82.0%) of cases, and the most common clinical feature was watery diarrhoea 119/123 (96.7%). Antimicrobials were discontinued in 53/105 (50.5%) cases and 118/123 (95.9%) of them received metronidazole as the initial treatment. The mean treatment duration (±SD) was 9.08 ± 5.6 days. Fifteen (12.7%) patients failed the first course of antimicrobial therapy, of which four were treated with oral vancomycin, and eleven patients received both drugs. Recurrence of infection was observed in 12/118 (10.2%) patients and 30-day mortality was 38/123 (30.9%). Several clinical variables were associated with increased 30-day mortality on univariate analysis. Only occurrence of disease among Qataris, prolonged hospitalisation, positive stool occult blood test, high white blood cells and septic shock were found to be independent predictors of mortality by multivariate logistic regression analysis. In conclusion, C. difficile infection was a recognise cause of morbidity and mortality in our hospital with low and stable incidence. It involved predominantly patients younger than 65 years with underlying illness and metronidazole and vancomycin were effective in resolving symptoms in the majority of our patients.