scholarly journals Haemophagocytic Lymphohistiocytosis in a Malay infant: Rare, Old and Often Forgotten Disease

2022 ◽  
Vol 21 (1) ◽  
pp. 196-200
Author(s):  
Norpazila Yaacob ◽  
Mohd Nazri Hassan ◽  
Faezahtul Arbaeyah Hussain ◽  
Rosnah Bahar ◽  
Ariffin Nasir ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Medical Science ◽  
Severe Anaemia ◽  
Clinical Syndrome ◽  
Recurrent Fever ◽  
Bleeding Tendency ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Neutropenic Sepsis ◽  
Familial Type ◽  
Life Threatening

Haemophagocytic lymphohistiocytosis (HLH) is a rare disease but potentially life threatening clinical syndrome. It is caused by a multisystemic hyperinflammatory process secondary to severe hypercytokinemia with excessive and uncontrolled activation of the immune response. We report a case of familial HLH with no apparent causes in 6 months-old Malay girl presented with recurrent fever associated with severe anaemia and bleeding tendency requiring extensive treatment but refractory to the treatment which lead to mortality due to neutropenic sepsis indicating of poor prognosis of this disease. This familial type of HLH should be suspected in all children after excluding all the secondary causes with collective laboratory features and requiring extensive management as it associated with high mortality. Bangladesh Journal of Medical Science Vol. 21(1) 2022 Page : 196-200

Haemophagocytic Lymphohistiocytosis (HLH) in a case of Enteric Fever

2016 ◽  
Vol 5 (03) ◽  
pp. 4882
Author(s):  
Vineeta Pande ◽  
Agarkhedkar S. R. ◽  
Ayank Tandon* ◽  
Aditya Agarwal
Keyword(s):  
Inflammatory Reaction ◽  
Enteric Fever ◽  
Infectious Agent ◽  
Clinical Syndrome ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Life Threatening ◽  
Stimulation Of

HLH is an uncommon, life threatening clinical syndrome cause by a severe hyper inflammatory reaction triggered by an infectious agent. The characteristic symptoms of HLH are due to the persistent stimulation of lymphocytes and histiocytes, leading to hyper-cytokinemia. We report a case of HLH in enteric fever in a13 year old female presenting with fever, lymphadenopathy and pancytopenia due an infection caused by Salmonella.

scholarly journals Risk stratification in cardiogenic shock: a focus on the available evidence

2021 ◽  
Author(s):  
C. Sciaccaluga ◽  
G. E. Mandoli ◽  
N. Ghionzoli ◽  
F. Anselmi ◽  
C. Sorini Dini ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Cardiogenic Shock ◽  
Clinical Syndrome ◽  
Diagnostic Assessment ◽  
Prognostic Scores ◽  
Current Evidence ◽  
Efficient Resource ◽  
Threatening Condition ◽  
Life Threatening ◽  
Future Direction

AbstractCardiogenic shock is a clinical syndrome which is defined as the presence of primary cardiac disorder that results in hypotension together with signs of organ hypoperfusion in the state of normovolaemia or hypervolaemia. It represents a complex life-threatening condition, characterized by a high mortality rate, that requires urgent diagnostic assessment as well as treatment; therefore, it is of paramount important to advocate for a thorough risk stratification. In fact, the early identification of patients that could benefit the most from more aggressive and invasive approaches could facilitate a more efficient resource allocation. This review attempts to critically analyse the current evidence on prognosis in cardiogenic shock, focusing in particular on clinical, laboratoristic and echocardiographic prognostic parameters. Furthermore, it focuses also on the available prognostic scores, highlighting the strengths and the possible pitfalls. Finally, it provides insights into future direction that could be followed in order to ameliorate risk stratification in this delicate subset of patients.

scholarly journals Symmetrical peripheral gangrene in a child following severe malaria with concomitant sepsis

2020 ◽  
Vol 47 (3) ◽  
pp. 280-284
Author(s):  
O.S. Katibi ◽  
B.S. Olorunshola ◽  
O.O. Akintade ◽  
O.S. Folayan
Keyword(s):  
Early Detection ◽  
Severe Malaria ◽  
Severe Anaemia ◽  
Clinical Syndrome ◽  
Ischemic Damage ◽  
Permanent Disability ◽  
Peripheral Gangrene ◽  
Distal Interphalangeal ◽  
Left And Right ◽  
High Index Of Suspicion

Symmetrical peripheral gangrene (SPG) is a welldocumented but rare clinical syndrome characterized by symmetrical distal ischemic damage  leading to gangrene of two or more sites in the absence of large vessel obstruction or vasculitis. The aetiological factors responsible for SPG are vast and it could follow many common diseases such as malaria. This is a report of a 9month old child who developed symmetric peripheral gangrene following severe malaria (severe anaemia) and sepsis. Gangrene involved the 2nd to the 5thdigits and 3rd and 4thdigits of the left and right hands and all the toes. Autoamputation of the affected digits followed several weeks after discharge and was limited to the distal  interphalangeal joints. There is the need for a high index of suspicion, early detection and prompt management of patients with disorders associated with SPG in order to limit the risk of permanent disability in otherwise treatable diseases. Key words: peripheral, gangrene, malaria, child

The patient presenting with decompensated cirrhosis

2013 ◽  
Vol 12 (4) ◽  
pp. 232-238
Author(s):  
Philip A Berry ◽  
◽  
Sam J Thomson ◽  
Keyword(s):  
Poor Prognosis ◽  
Alcoholic Hepatitis ◽  
Early Recognition ◽  
Kidney Injury ◽  
Decompensated Cirrhosis ◽  
Organ Support ◽  
Early Management ◽  
Related Disease ◽  
Life Threatening ◽  
The Uk

The rates of liver disease in the UK are rising and hence more patients than ever are presenting to acute medical units with potentially life threatening sequelae. Early recognition and treatment of sepsis, kidney injury, bleeding and alcoholic hepatitis can significantly improve outcomes, but requires a comprehensive approach to assessment. This patient cohort often suffers from a perceived uniform poor prognosis, especially in alcohol related disease, but evidence for this is changing and reassessment of prognosis after 48 hours of organ support may be more accurate than that made ‘at the front door’. This article summarises the most important complications of decompensated cirrhosis, their early management, and presents a targeted system of care: ‘RING Liver’ – Renal failure, Infection, Nutrition, Gastrointestinal bleeding and transit, Liver dysfunction/transplantation. Factors favouring transfer to tertiary units are also explored.

Haemophagocytic lymphohistiocytosis secondary to brucellosis in a young child

2021 ◽  
Vol 14 (3) ◽  
pp. e240759
Author(s):  
Jashan Mittal ◽  
Prawin Kumar ◽  
Jagdish Prasad Goyal ◽  
Abhishek Purohit
Keyword(s):  
Haemophagocytic Lymphohistiocytosis ◽  
Appetite Loss ◽  
Specific Therapy ◽  
Adequate Treatment ◽  
Underlying Condition ◽  
Persistent Fever ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of ◽  
Life Threatening Complication

Brucellosis is a common zoonotic disease worldwide. It has protean clinical manifestation and sometimes may has a life-threatening complication. A 4-year-old boy presented with a history of fever, myalgia and appetite loss for 3 weeks. On examination, he had hepatosplenomegaly. The initial working diagnosis was an infection, autoimmune disease and malignancy. Investigations showed positive Brucella serology, and he was started on rifampicin and cotrimoxazole. He was further investigated because of persistent fever, which revealed evidence of haemophagocytic lymphohistiocytosis (HLH). He continued treatment for brucellosis, except rifampicin which was replaced with doxycyclin due to a worsening liver function. The child showed complete clinical and biochemical improvement after 6 weeks of therapy. HLH is a life-threatening condition and should be suspected in children with brucellosis, who did not respond to appropriate antibiotics treatment. Secondary HLH does not always require specific therapy; it may improve with adequate treatment of the underlying condition.

Prediction Models

2021 ◽  
pp. 50-69
Author(s):  
Amit Kumar Tyagi
Keyword(s):  
Predictive Modeling ◽  
Prediction Models ◽  
Medical Science ◽  
Rapid Change ◽  
Well Being ◽  
Smart Devices ◽  
Prediction Modeling ◽  
Early Stages ◽  
Life Threatening

Having/living convenient life through smart devices, people are more interested or more dependent on to predict something for future (i.e., with respect to their health, business, etc.). For that, many prediction models by several researchers are being used in many applications. Due to a vast (rapid) change in lifestyle, people are more prone to a number of life-threatening diseases when it comes to their well-being. Many of these diseases start developing their symptoms in their early stages. But, still many of these diseases like cancer, kidney damages remain unidentified in their developing stages. The earlier a disease is predicted, the easier it becomes to cure it and even prevent it. Predictive modeling provides a huge step forward in medical science in preventing the risk among patients. Prediction modeling is the process of analyzing current conditions to predict future results.

scholarly journals Risks associated with oral deferiprone in the treatment of infratentorial superficial siderosis

2019 ◽  
Vol 267 (1) ◽  
pp. 239-243 ◽  
Author(s):  
Y. Sammaraiee ◽  
G. Banerjee ◽  
S. Farmer ◽  
B. Hylton ◽  
P. Cowley ◽  
...  
Keyword(s):  
Side Effects ◽  
Case Series ◽  
Iron Chelator ◽  
Superficial Siderosis ◽  
Neutropenic Sepsis ◽  
Favourable Safety ◽  
Favourable Safety Profile ◽  
Life Threatening ◽  
The Mean ◽  
Mean Time

Abstract Objective Deferiprone is an iron chelator that has recently been used to treat patients with infratentorial superficial siderosis (iSS). It is considered to have a generally favourable safety profile but concerns have been raised due to the risk of agranulocytosis. We aimed to evaluate the safety and tolerability of oral deferiprone as a treatment for patients with iSS. Methods We present a case series of 10 consecutive patients presenting with classical iSS treated with deferiprone. Results Ten patients were followed up for a mean period of 2.3 years (range 0.5–5.5 years). Four patients (40%) were withdrawn from treatment because of treatment-related side effects. The reasons for treatment discontinuation were neutropenic sepsis (n = 3) and fatigue (n = 1). In 2 out of the 3 cases of neutropenic sepsis, patients initially developed neutropenia without sepsis. The mean time to neutropenic sepsis following deferiprone was 1.2 years (range 0.3–2.5) with mean neutrophil count of 0.4 (range 0.3–0.5). Six patients (60%) reported no change in neurological function while on treatment, and four patients (40%) reported that their condition deteriorated. Conclusions Deferiprone was poorly tolerated, with 40% of patients withdrawing from treatment, most commonly due to neutropenic sepsis, after an average of 2 years on treatment. This study increases the number of reported cases of agranulocytosis in patients with iSS treated with deferiprone. Clinicians treating iSS patients with deferiprone should be aware that this drug has a potentially life-threatening side effect of neutropenic sepsis, and should ensure that appropriate haematological monitoring is in place.

scholarly journals Risk-Benefit Events Associated with the Use of Aspirin for Primary Prevention of Cardiovascular Disorders

2020 ◽  
Author(s):  
Deepak Kumar Dash ◽  
Vishal Jain ◽  
Anil Kumar Sahu ◽  
Rajnikant Panik ◽  
Vaibhav Tripathi
Keyword(s):  
Primary Prevention ◽  
Adverse Drug Events ◽  
Medical Science ◽  
Therapeutic Effects ◽  
Cardiovascular Disorders ◽  
Drug Of Choice ◽  
Administration Regimen ◽  
Life Threatening ◽  
Cvd Prevention ◽  
Prevention Of Cardiovascular Disease

Aspirin had been introduced as a nonsteroidal anti-inflammatory molecule. As further research on aspirin started, other therapeutic effects have been revealed. Now, this molecule has become the polychrest in medical science. Aspirin has served as a drug of choice for the primary prevention of cardiovascular disease (CVD) for the last few decades. However, recent trials have raised questions on the use of aspirin for CVD prevention due to some life-threatening adverse drug events. In spite of that, outcomes of trials will surely assist to frame a guideline for anoxic administration regimen of aspirin in order to prevent CVD.

Intensified concomitant chemoradiotherapy with and without filgrastim for poor-prognosis head and neck cancer.

1994 ◽  
Vol 12 (11) ◽  
pp. 2351-2359 ◽  
Author(s):  
E E Vokes ◽  
D J Haraf ◽  
R Mick ◽  
J M McEvilly ◽  
R R Weichselbaum
Keyword(s):  
Head And Neck Cancer ◽  
Head And Neck ◽  
Neck Cancer ◽  
Poor Prognosis ◽  
Locoregional Control ◽  
Cumulative Toxicity ◽  
Life Threatening ◽  
Group 2 ◽  
Concomitant Radiotherapy ◽  
Group 1

PURPOSE We previously demonstrated high locoregional control rates in patients with poor-prognosis head and neck cancer using fluorouracil (5-FU), hydroxyurea (HU), and concomitant radiotherapy (FHX). In two trials reported here, we added cisplatin with and without granulocyte colony-stimulating factor (G-CSF) to 5-FU, HU, and concomitant radiotherapy. PATIENTS AND METHODS Eligible patients had failed to respond to prior local therapy (group 1); previously untreated patients with unresectable and/or metastatic disease and a projected 2-year survival rate less than 10% were also eligible (group 2). Chemoradiotherapy consisted of 1.8 to 2.0 Gy on days 1 to 5 with simultaneous infusional 5-FU at 800 mg/m2/d and HU administered every 12 hours for 11 doses at escalating doses. Cisplatin was administered at 100 mg/m2 during every other cycle. Cycles were repeated every 14 days until completion of radiotherapy. In study 2, G-CSF was added on days 6 to 13 at 5 micrograms/kg/d. RESULTS Acute and cumulative myelosuppression limited the feasibility of adding cisplatin to FHX without G-CSF. G-CSF allowed for escalation of HU to 1 g orally every 12 hours without dose-limiting acute toxicity during cycles 1 and 2. Dose-limiting cumulative toxicity consisted of severe or life-threatening myelosuppression and mucositis. To decrease total treatment duration and, thus, cumulative toxicity, a hyperfractionated radiation therapy schedule was investigated using the established chemotherapy doses with 1.5 Gy twice daily on days 1 to 5 (75 Gy over five treatment cycles). No increase in acute toxicities was seen; cumulative toxicities remained frequently severe or life-threatening. Thirty-eight of 45 assessable patients responded. The median survival duration was 12 months for both groups. Median time to treatment failure was 8 months for group 1 and has not been reached for group 2. At 1 year, local control rates were 74% and 91% for groups 1 and 2, respectively. CONCLUSION The addition of cisplatin to 5-FU, HU, and concomitant radiotherapy is feasible using G-CSF. The high locoregional control rate and failure-free interval justify further investigation of this regimen in previously untreated patients.

scholarly journals Staphylococcal Panton–Valentine Leucocidin and Gamma Haemolysin Target and Lyse Mature Bone Marrow Leucocytes

Toxins ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 725
Author(s):  
Elisabeth Hodille ◽  
Adriana Plesa ◽  
Eve Bourrelly ◽  
Lucie Belmont ◽  
Cédric Badiou ◽  
...  
Keyword(s):  
Staphylococcus Aureus ◽  
Bone Marrow ◽  
Poor Prognosis ◽  
Staphylococcal Infection ◽  
Host Susceptibility ◽  
Human Pathogen ◽  
C5ar Expression ◽  
Life Threatening ◽  
Immature Cells ◽  
Mature Bone

Staphylococcus aureus is a major human pathogen, inducing several infections ranging from the benign to the life-threatening, such as necrotising pneumonia. S. aureus is capable of producing a great variety of virulence factors, such as bicomponent pore-forming leucocidin, which take part in the physiopathology of staphylococcal infection. In necrotising pneumonia, Panton–Valentine leucocidin (PVL) induces not only lung injury and necrosis, but also leukopenia, regarded as a major factor of a poor prognosis. The aim of the present study was to evaluate the effect of bicomponent pore-forming leucocidin, PVL and gamma haemolysin on bone marrow leucocytes, to better understand the origin of leukopenia. Using multi-parameter cytometry, the expression of leucocidin receptors (C5aR, CXCR1, CXCR2, and CCR2) was assessed and toxin-induced lysis was measured for each bone marrow leucocyte population. We observed that PVL resulted in myeloid-derived cells lysis according to their maturation and their C5aR expression; it also induced monocytes lysis according to host susceptibility. Haemolysin gamma A, B, and C (HlgABC) displayed cytotoxicity to monocytes and natural killer cells, hypothetically through CXCR2 and CXCR1 receptors, respectively. Taken together, the data suggest that PVL and HlgABC can lyse bone marrow leucocytes. Nevertheless, the origin of leukopenia in severe staphylococcal infection is predominantly peripheral, since immature cells stay insensitive to leucocidins.

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