Hemoglobinopathies and Thalassemias
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
Children and adults with sickle cell diseases are prone to pulmonary complications referred to as the acute chest syndrome (ACS). An epidemiologic study has shown that new infiltrates occur in 12.8 per 100 patient-years. In young children the ACS was more apt to be due to infection and is seldom associated with pain. In older children and adults concomitant rib infarction, demonstrable by radionuclide bone scintigraphy in 50% of cases, is the primary event. Presumably the inflammation surrounding infarcts in ribs creates pleuritis, splinting, atelectasis, hypoxemia, and increased sickling. The clinical picture may rapidly proceed to adult respiratory distress syndrome or multiorgan failure. (See below for therapy.) The number of episodes of ACS determines life expectancy. Severe episodes of bone infarction may result in embolism of necrotic marrow into the lungs, brain, kidney, and other organs. It is difficult to recognize and is usually fatal.
Acute pain and medical disorders
Pamela E Macintyre, Suellen M Walker, David J Rowbotham in Clinical Pain Management, 2008
A sickle cell crisis most commonly affects multiple bony sites or joints (due to avascular necrosis of bone marrow) resulting in severe pain lasting for hours to weeks, usually in the low back, legs, knees, arms, or chest.197 Crises involving abdominal organs, such as the spleen, gut, liver, or gallbladder may present as an acute surgical abdomen. Priapism is not uncommon and renal colic may develop due to renal papillary necrosis and hematuria. An “acute chest syndrome” presents with chest pain, cough, dyspnea, and fever with hypoxemia, leukocytosis, and pulmonary infiltrates on a chest radiograph. Other causes of acute pain include ischemic leg ulcers or central pain associated with stroke or spinal cord infarction.
The Pale Child
Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan in Diagnosing and Treating Common Problems in Paediatrics, 2017
Acute chest syndrome is the second most frequent cause of admission to hospital of children with sickle-cell disease. The aetiologies include bacteria (pneumococcus, Gram-negative bacteria, chlamydia and Mycoplasma pneumoniae) and viruses (respiratory syncytial virus, influenza and parainfluenza). Non-infectious causes include pulmonary infarction, pulmonary oedema from fluid overload, hypoventilation from pain or narcotic administration. Children under 4 years of age present with cough fever and upper lobe disease. Older children present with dyspnoea, chills, no fever and multiple lobes involved (inclusive of lower lobe) on chest X-ray. Tenderness over the ribs is frequently noted.
Successful Use of Veno-Venous Extracorporeal Membrane Oxygenation in an Adult Patient with Sickle Cell Anemia and Severe Acute Chest Syndrome
Published in Hemoglobin, 2018
Sarah S. Sewaralthahab, Jay Menaker, Jennie Y. Law
Sickle cell disease is an inherited hemoglobinopathy affecting an estimated 100,000 individuals in the United States, of whom 60.0% are adults [1]. Life expectancy for adult patients with sickle cell disease is 25-30 years lower than the general population with morbidity increased by the risk of vaso-occlusive crises (VOCs), acute chest syndrome (ACS), and cerebral vascular infarcts [2]. Acute chest syndrome is a life threatening pulmonary complication of sickle cell disease consisting of pulmonary infiltrates with associated leukocytosis, fever, cough, chest pain and dyspnea. Severe cases of ACS are associated with profound hypoxia and often require ventilatory support. Acute chest syndrome remains the leading cause of death in adult patients with sickle cell disease, resulting in approximately 25.0% of the mortality due to sickle cell disease [3] and is the second most frequent cause of hospitalization after a VOC episode [2]. Acute chest syndrome can be precipitated by atelectasis due to rib or vertebral infarcts, infection, occlusion of pulmonary vasculature, and resultant infarction. These may often coexist making it difficult to establish a single cause [4]. Treatment of ACS includes pain management, hydration, antibiotic therapy, and aggressive respiratory support. In severe cases, red blood cell (RBC) exchange is used to both improve oxygenation and prevent further damage by the sickled RBCs. Despite these measures, many patients will require intubation to aid in gas exchange given development of acute respiratory distress (ARDS).
Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Outcomes. The Experience of a Single Thalassemia and Sickle Cell Unit in a University Hospital
Published in Hemoglobin, 2021
Sophia Delicou, Konstantina Aggeli, Konstantinos Magganas, Dimitrios Patsourakos, Aikaterini Xydaki, John Koskinas
Acute chest syndrome is a complication of sickle cell disease characterized by pleuritic chest pain, fever, rales and chest X-ray pulmonary infiltrations. In 1979, Charache et al. [1] first proposed the use of the word “acute chest syndrome” for this complication, recognizing the difficulties in evaluating its pathogenesis. Acute chest syndrome occurs in as many as half of sickle cell disease patients [2], is among the primary causes of hospitalization and a potential cause of death. In the sickling conditions, the pathophysiology of acute lung injury is complex. This process can be caused by microbial or viral infection, in situ vaso-occlusion, fat embolisms from ischemic/necrotic bone marrow or thromboembolism, and it is often difficult to determine a definite cause. Important factors in ACS pathogenesis include functional asplenia, decreased serum opsonic activity and relatively poor antibody response to the bacteria’s capsule polysaccharide component [3].
Asthmonia: A clinical definition of a commonly used colloquial term
Published in Journal of Asthma, 2018
Caleb D. Wasser, Anna Grushevsky, Stephanie T. Johnson, Sharon R. Smith
Medical diagnoses are constantly evolving as evidenced by Sir William Osler's 1892 definition of asthma as a neurotic affection compared to the more recent definition of asthma as chronic inflammation with constriction (6). With time, further clinical research and pharmaceutical developments, advancements are made to better elicit medical entities and their therapies. With this progress, we need to consider if the colloquial use of terms such as asthmonia, bear weight in influencing medical care. The overlap of asthma with chronic obstructive pulmonary disease lead to a descriptive paper regarding therapeutic options for this newly defined syndrome (7). Another example of a syndrome defined by the intersection of two disease entities is acute chest syndrome; sickle cell pain crisis with concurrent new infiltrate on chest radiograph (8). There is an accepted definition of acute chest syndrome that includes a new infiltrate, chest pain, fever, hypoxia and respiratory symptoms. Although the etiology of acute chest syndrome is complex, infections have been documented in about 1/3 of cases. Management for acute chest syndrome is now recognized as requiring therapy for sickle cell pain crises, respiratory support as necessary and broad spectrum antibiotics (9). We believe that management of asthmonia will also require a multifactorial approach in proper management as well.
Related Knowledge Centers
- Infiltration
- Inflammation
- Shortness of Breath
- Hypoxia
- Lung
- Circulatory System
- Vaso-Occlusive Crisis
- Sickle Cell Disease
- X-Ray
- Sputum
- Shortness of Breath