scholarly journals P104 A review of patients referred to Rheumatology with haemophagocytic lymphohistiocytosis who were treated with anakinra successfully

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Dominik Kurzeja ◽  
James Perera ◽  
Kavya Pillai ◽  
Sanaah Amin ◽  
Richard Stratton ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Interleukin 1 ◽  
Treatment Strategies ◽  
Primary Diagnosis ◽  
Royal Free Hospital ◽  
Female Ratio ◽  
Hospital Patients ◽  
Threatening Condition ◽  
Life Threatening ◽  
Poor Outcomes

Abstract Background/Aims  Haemophagocytic lymphohistiocytos (HLH) is a potentially life-threatening condition characterised by over-activation of the innate immune system. It can be classified into primary HLH, often inherited, or secondary which is triggered by non-genetic causes, such as chronic immunosuppression, malignancy or infection.Primary HLH is more frequently observed in infants, however, secondary HLH is more commonly observed in adults. Diagnosis is often delayed, most likely due to the rarity of the condition and is usually prompted by serological abnormalities, including hyperferritinaemia, organ and bone marrow dysfunction.Treatment options are limited; however, the Royal Free Hospital has observed a number of secondary HLH cases who were successfully treated with steroids and anakinra. Methods  A retrospective search of clinic letters and patient records was undertaken using electronic care records. We also searched through pharmacy records of patients treated with anakinra at the Royal Free Hospital. Patients with a documented diagnosis of HLH prior to March 2020 who had been referred to Rheumatology were included. We excluded patients after March 2020 due to patients with COVID-19 having similar overlap of symptoms and biomarkers as those with HLH. We identified key demographic details, laboratory, pathology investigations,the course of hospital admission, primary diagnosis and follow-up data. The diagnosis was confirmed withan H-score and bone marrow biopsy where possible. Results  We identified nine adult cases with adocumented diagnosis of HLH whohad been admitted to the Royal Free Hospitaland referred to Rheumatology. They had allbeen subsequently followed up in outpatientclinic.Ages of patients ranged from 19 to 58 with a male to female ratio of 2:1. The predominant underlying pathology was adultonsetStill’s disease(78%), with the remaining cases being secondary to HIV (22%). Bone marrow aspirate was performed in all patients and 78% of patients aspirated had bone marrowappearances in keeping with HLH.Ferritin levels varied from 532 micrograms/Lto 93309 micrograms/L with a median ferritin of 5045.5 micrograms/L.H-Scores were variable witha median score of 171.5 (96-238).All patients received steroids as part of their treatment. Anakinra (interleukin-1 receptor antagonist) was delivered intravenously(6/9) or subcutaneously (3/9). Conclusion  We present data from nine cases of HLH treated with anakinra who survived their admission and have been subsequently followed up in clinic. We found that the majority of these patients had an underlying diagnosis of adult onset Still’s disease. Anakinra was used successfully as part of their treatment both intravenously as well as subcutaneously. Further work is needed comparing patients with good and poor outcomes to establish identifiable prognostic markers as well as effective treatment strategies. Disclosure  D. Kurzeja: None. J. Perera: None. K. Pillai: None. S. Amin: None. R. Stratton: None. A. Singh: None.

scholarly journals Management of ST Elevation Myocardial Infarction (STEMI) in Different Settings

2021 ◽  
Author(s):  
Rod Partow-Navid ◽  
Narut Prasitlumkum ◽  
Ashish Mukherjee ◽  
Padmini Varadarajan ◽  
Ramdas G. Pai
Keyword(s):  
Myocardial Infarction ◽  
Treatment Strategies ◽  
Management Process ◽  
St Segment Elevation ◽  
Segment Elevation Myocardial Infarction ◽  
Multiple Factors ◽  
St Segment ◽  
Threatening Condition ◽  
Life Threatening ◽  
St Elevation

AbstractST-segment elevation myocardial infarction (STEMI) is a life-threatening condition that requires emergent, complex, well-coordinated treatment. Although the primary goal of treatment is simple to describe—reperfusion as quickly as possible—the management process is complicated and is affected by multiple factors including location, patient, and practitioner characteristics. Hence, this narrative review will discuss the recommended management and treatment strategies of STEMI in the circumstances.

scholarly journals Cervical Pregnancy: Two Cases with Different Treatment Strategies

2016 ◽  
Vol 6 (1) ◽  
pp. 51-54
Author(s):  
Hanan M. Shamrani
Keyword(s):  
Therapeutic Option ◽  
Treatment Strategies ◽  
First Trimester ◽  
Management Options ◽  
Uterine Preservation ◽  
Cervical Pregnancy ◽  
Dilatation And Curettage ◽  
First Case ◽  
Threatening Condition ◽  
Life Threatening

Cervical pregnancy is a rare, potentially life-threatening condition that presents challenging management options. We report the cases of two patients: a 38-year-old, gravida 11 para 8+ 2 who presented with first-trimester vaginal bleeding and a 32-year-old female primigravida who presented in her ninth week of gestation with vaginal spotting and abdominal pain. Radiological and histopathological findings were consistent with cervical pregnancy in both cases, which were managed successfully with different approaches. Conservative management with intra-embryonic injection of potassium chloride was successful in the first case while dilatation and curettage after intramuscular methotrexate administration resolved the second patient's symptoms. Cervical pregnancy, when diagnosed early, can be successfully treated with medical therapy. Surgical intervention may be necessary, but adopting medical treatment as a first-line therapeutic option offers the advantage of uterine preservation.

Diagnostic Challenges And Clinical Implications For Systemic EBV-Associated T-Cell Lymphoproliferative Disorders Of Childhood

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S158-S158
Author(s):  
C Yasa Benkli ◽  
A Marcogliese ◽  
M Elghetany ◽  
J Punia ◽  
K Fisher ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Treatment Strategies ◽  
Marrow Transplant ◽  
Lymphoproliferative Disorders ◽  
Architectural Distortion ◽  
Clinicopathologic Findings ◽  
Children And Young Adults ◽  
Poor Outcomes

Abstract Introduction/Objective Systemic EBV-associated T-cell lymphoproliferative disorders of childhood (S-EBV-T-LPD) comprise three major forms: EBV-positive hemophagocytic lymphohistiocytosis (EBV-HLH), systemic EBV-positive T- cell lymphoma (S-EBV-TCL), and systemic T-cell chronic active EBV infection (S-T-CAEBV). These disorders are rare in children and young adults in Western countries and are associated with poor outcomes. Frequently patients were treated initially for EBV-HLH and subsequently found to have relapsed/refractory EBV-HLH vs S-EBV-TCL or overt EBV+ TCL, the latter of which requires different therapy than EBV-HLH. We report the clinicopathologic findings of 13 cases, including 8 previously reported. (PMID: 31099136) Methods Thirteen cases of S-EBV-T-LPD were identified at Texas Children’s Hospital from 1990 to 2020. Clinicopathologic and relevant laboratory parameters were recorded. Results Patients included six females and seven males of Hispanic (n=6), Asian (4), and Caucasian origins (3) ages 1-22 years (median 2). All had fever, hepatosplenomegaly, cytopenias, abnormal EBV serologies, and significantly elevated peripheral blood EBV- DNA load by quantitative PCR. Histologic features were variable ranging from EBV+ T-cell infiltrates with subtle architectural distortion and mild atypia to overt morphologic appearance of lymphoma. Consistent findings were aberrant T-cell populations identified by immunohistochemistry (n=4) or bone marrow flow cytometry (n=9, 1-50%, mean 16%). Five patients were classified as overt EBV+ TCL with abnormal karyotyping identified in 3. Seven patients had pathology findings indistinguishable between EBV-HLH and S-EBV-TCL and were classified as EBV-HLH/S-EBV-TCL. One patient had S-T-CAEBV. Outcomes were dismal after HLH-directed immuno/chemotherapy protocols with/without bone marrow transplant as only three EBV-HLH/S-EBV-TCL patients were alive at 1.5, 2.5 months, and 9 years follow-up. Conclusion This series from North America demonstrate challenges in the diagnosis and management of S-EBV-T- LPD cases. Particularly, EBV-HLH and S-EBV-TCL, which require vastly different treatment strategies, may initially present with overlapping clinicopathological features. Further studies are needed to address clear diagnostic criteria to guide appropriate management.

scholarly journals The Contribution of Host Cells to Pneumocystis Immunity: An Update

Pathogens ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 52 ◽  
Author(s):  
Patricia Otieno-Odhiambo ◽  
Sean Wasserman ◽  
J. Claire Hoving
Keyword(s):  
Treatment Strategies ◽  
Pneumocystis Pneumonia ◽  
Host Cells ◽  
Causative Organism ◽  
Aids Patients ◽  
Host Immune Responses ◽  
Life Threatening ◽  
New Treatment ◽  
Poor Outcomes ◽  
Immunodeficiency Syndrome

Pneumocystis is a ubiquitous atypical fungus that is distributed globally. The genus comprises morphologically similar but genetically heterogeneous species that have co-evolved with specific mammalian hosts as obligate intra-pulmonary pathogens. In humans, Pneumocystis jirovecii is the causative organism of Pneumocystis pneumonia (PCP) in immunocompromised individuals, a serious illness frequently leading to life-threatening respiratory failure. Initially observed in acquired immunodeficiency syndrome (AIDS) patients, PCP is increasingly observed in immunocompromised non-AIDS patients. The evolving epidemiology and persistently poor outcomes of this common infection will require new strategies for diagnosis and treatment. A deeper understanding of host immune responses and of the cells that mediate them will improve the chance of developing new treatment strategies. This brief review provides an update on recent studies on the role of host immunity against Pneumocystis.

Case of using mycophenolate in combination with steroids for concurrent macrophage activation syndrome and lupus flare

2020 ◽  
Vol 13 (4) ◽  
pp. e231554
Author(s):  
Kavina Shah ◽  
Andrew Porter ◽  
Gagandeep Takhar ◽  
Venkat Reddy
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Treatment Strategies ◽  
Systemic Lupus ◽  
Severe Systemic Lupus Erythematosus ◽  
Threatening Condition ◽  
Life Threatening ◽  
Activation Syndrome

This report highlights the importance of tailored treatment strategies in severe systemic lupus erythematosus (SLE) flares driving the life-threatening condition, macrophage activation syndrome (MAS). We report the case of a 42-year-old woman with active systemic lupus erythematosus (SLE) who was diagnosed with MAS within 3 days of onset of lethargy, rash, joint pain and significant cytopenias. This early diagnosis meant that her condition was managed with less intensive immunosuppression with only modest doses of steroids and mycophenolate mofetil.

Analysis of Causes and Outcome of Thrombocytopenia as a Presenting Symptom in a Single Institution.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3962-3962
Author(s):  
Doris M. Kraemer ◽  
Barbara Bauer ◽  
Joerg C. Rath ◽  
Hermann Einsele
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Rectal Carcinoma ◽  
Bone Marrow Failure ◽  
Rare Event ◽  
Treatment Strategies ◽  
Primary Diagnosis ◽  
Severe Bleeding ◽  
Severe Thrombocytopenia ◽  
Adequate Treatment

Abstract Patients suffering from thrombocytopenia admitted to the emergency room during nights and weekends might be a problem, depending from the availability of a haematologist on duty. We evaluated retrospectively the medical history of all patients entering our department receiving a bone marrow biopsy due to severe thrombocytopenia in the years from 2000–2003. We analyzed 441 patients admitted due to thrombocytopenia. 146 patients had a platelet count < 50,000/μl. 69 of these patients suffered from severe thrombocytopenia < 20,000/μl. All patients entering the ER during daytime received a bone marrow biopsy during the first three hours post admission. If patients were admitted during the night, they received a biopsy the following morning. Bone marrow smears were stained according to Pappenheim and evaluated as soon as they were dry. Diagnoses were as the following: acute leukaemia 54; immune thrombocytopenia 31; aplastic anaemia/toxic bone marrow failure/myelodysplastic syndrome 36; Non Hodgkin’s Lymphoma 7; carcinosis of bone marrow 8; myeloproliferative syndrome 3; viral infection (Hanta, HIV) 2; sepsis 1; hypersplenism 2; thrombotic thrombocytopenic purpura 1; heparin induced thrombocytopenia type II 1. During their time in the hospital only one patient of the 146 patients developed severe bleeding with the need of transfusion of blood packs. The 73 y old male patient with primary diagnosis of rectal carcinoma and 23,000 platelets at admission was diagnosed with secondary acute myeloid leukaemia. He showed lower gastrointestinal bleeding since rectal carcinoma could not be treated by surgery due to the leukaemia. In summary 1 out of 146 patients with severe thrombocytopenia showed clinical bleedings (<1%), indicating this as a very rare event. Due to the fact that only 31 out of 146 patients (or 20 of 69 patients with platelets < 20,000/μl) with severe thrombocytopenia suffered from immune mediated thrombocytopenia immediate consultation of the haematologist is essential to allow rapid diagnosis and adequate treatment strategies in patients presenting with thrombocytopenia in the emergency department.

Update on Infections in the Immunocompromised Host

1991 ◽  
Vol 4 (5) ◽  
pp. 282-294
Author(s):  
C. Randall Marchbanks ◽  
Karen A. Rowley
Keyword(s):  
Bone Marrow ◽  
Medical Care ◽  
Treatment Strategies ◽  
Immunocompromised Host ◽  
Immunocompromised Patients ◽  
Novel Treatment ◽  
Recent Advances ◽  
Life Threatening ◽  
Immunocompromised State ◽  
Novel Treatment Strategies

Recent advances in medical care have provided more effective therapies for the treatment of various malignancies and increased the number of successful bone marrow and organ transplantations. However, these advances often place the patient in a severely immunocompromised state for several days or weeks resulting in one or more life-threatening infections. This article discusses some general principles, current pharmacotherapeutic strategies, and novel treatment strategies for the management of immunocompromised patients.

Intracranial hypotension producing reversible coma: a systematic review, including three new cases

2012 ◽  
Vol 117 (3) ◽  
pp. 615-628 ◽  
Author(s):  
Joshua J. Loya ◽  
Stefan A. Mindea ◽  
Hong Yu ◽  
Chitra Venkatasubramanian ◽  
Steven D. Chang ◽  
...  
Keyword(s):  
Intracranial Hypotension ◽  
Diagnosis And Treatment ◽  
Comatose Patient ◽  
Head Ct ◽  
Neurological Improvement ◽  
Management Algorithm ◽  
Csf Leakage ◽  
Threatening Condition ◽  
Life Threatening ◽  
Poor Outcomes

Intracranial hypotension is a disorder of CSF hypovolemia due to iatrogenic or spontaneous spinal CSF leakage. Rarely, positional headaches may progress to coma, with frequent misdiagnosis. The authors review reported cases of verified intracranial hypotension–associated coma, including 3 previously unpublished cases, totaling 29. Most patients presented with headache prior to neurological deterioration, with positional symptoms elicited in almost half. Eight patients had recently undergone a spinal procedure such as lumbar drainage. Diagnostic workup almost always began with a head CT scan. Subdural collections were present in 86%; however, intracranial hypotension was frequently unrecognized as the underlying cause. Twelve patients underwent one or more procedures to evacuate the collections, sometimes with transiently improved mental status. However, no patient experienced lasting neurological improvement after subdural fluid evacuation alone, and some deteriorated further. Intracranial hypotension was diagnosed in most patients via MRI studies, which were often obtained due to failure to improve after subdural hematoma (SDH) evacuation. Once the diagnosis of intracranial hypotension was made, placement of epidural blood patches was curative in 85% of patients. Twenty-seven patients (93%) experienced favorable outcomes after diagnosis and treatment; 1 patient died, and 1 patient had a morbid outcome secondary to duret hemorrhages. The literature review revealed that numerous additional patients with clinical histories consistent with intracranial hypotension but no radiological confirmation developed SDH following a spinal procedure. Several such patients experienced poor outcomes, and there were multiple deaths. To facilitate recognition of this treatable but potentially life-threatening condition, the authors propose criteria that should prompt intracranial hypotension workup in the comatose patient and present a stepwise management algorithm to guide the appropriate diagnosis and treatment of these patients.

Helplessness, Hope, and the Occupational Therapist

1977 ◽  
Vol 44 (2) ◽  
pp. 65-69 ◽  
Author(s):  
Joyce Magill ◽  
J. W. Vargo
Keyword(s):  
Perceived Control ◽  
Treatment Strategies ◽  
Specific Treatment ◽  
Occupational Therapist ◽  
Older Patient ◽  
Threatening Condition ◽  
Life Threatening ◽  
Anxiety Depression

This article examines how the loss of perceived control over one's environment (i.e. “helplessness”) can result in anxiety, depression, or death in people who otherwise appear to have favorable prognoses. Particular reference is made to the role of helplessness as a potentially life threatening condition in the older patient. Four effects of helplessness are discussed along with their implications for treatment. The paper presents seven specific treatment strategies for combatting the condition of helplessness, all of which are compatible with the skills possessed by an occupational therapist.

May-Thurner syndrome – Are we aware enough?

VASA ◽  
2019 ◽  
Vol 48 (5) ◽  
pp. 381-388 ◽  
Author(s):  
Katalin Mako ◽  
Attila Puskas
Keyword(s):  
Contraceptive Use ◽  
Iliac Vein ◽  
Common Iliac Vein ◽  
Vein Thrombosis ◽  
Compression Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
Iliac Vein Compression ◽  
Iliac Vein Compression Syndrome ◽  
The Right

Summary. Iliac vein compression syndrome (May-Thurner syndrome – MTS) is an anatomically variable clinical condition in which the left common iliac vein is compressed between the right common iliac artery and the underlying spine. This anatomic variant results in an increased incidence of left iliac or iliofemoral vein thrombosis. It predominantly affects young women in the second or third decades of life with preponderance during pregnancy or oral contraceptive use. Although MTS is rare, its true prevalence is underestimated but it can be a life-threatening condition due to development of pulmonary embolism (PE). In this case based review the authors present three cases of MTS. All patients had been previously confirmed with PE, but despite they were admitted to hospital, diagnosed and correctly treated for PE and investigated for thrombophilia, the iliac vein compression syndrome was not suspected or investigated. With this presentation the authors would like to emphasize that MTS is mostly underdiagnosed, and it needs to be ruled out in left iliofemoral vein thrombosis in young individuals.

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