Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature

Sheikh, Abu Baker; Nasrullah, Adeel; Lopez, Erick Daniel; Din, Mian Tanveer Ud; Sagheer, Shazib; Shah, Ishan; Javed, Nismat; Shekhar, Rahul

doi:10.1159/000519101

Cited by 9 publications

(10 citation statements)

References 32 publications

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“…The only medication that has been successfully demonstrated in prior studies to lower the frequency of painful episodes, rate of acute chest syndrome (ACS), and blood transfusions by 50% in adults is hydroxyurea, which is a myelosuppressive agent. It raises the levels of fetal Hb and Hb and was first tested in SCD in 1984 [ 11 , 12 ]. This group of patients should be informed about the symptoms of CL, its risk, and related comorbidities, as well as the necessity for routine preventative measures and regular follow-up because the incidence of CL in SCD is age-dependent.…”

Section: Discussionmentioning

confidence: 99%

Sickle Cell Disease (SCD) Leading to Pulmonary Arterial Hypertension (PAH) and Cholelithiasis (CL)

Chacko¹,

Jadhav²,

Ghewade³

et al. 2023

Cureus

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Sickle cell disease (SCD) consists of a variety of hereditary hemoglobinopathies linked to alterations in the beta component of the hemoglobin (Hb) molecule. Acute SCD manifestations include stroke, acute chest syndrome (ACS), and pain, whereas chronic manifestations include avascular necrosis, chronic renal disease, and gallstones. This case report describes a rare instance of SCD-related pulmonary arterial hypertension (PAH) and cholelithiasis (CL). Following investigations, such as high-resolution CT scan thorax, chest X-ray, two-dimensional echocardiography, and ultrasonography of the abdomen and pelvis, PAH and CL were confirmed. The medical intervention mainly involved oxygenation, IV fluids, IV antibiotics, simple packed red blood cell transfusion (SBCT), folic acid, calcium supplementation, hydroxyurea, chest physiotherapy, and respiratory muscle strengthening exercises. The surgical intervention for CL was planned. Hence, the learning point from this case is that early multidisciplinary approach should be taken in order to control the progression of SCD.

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Section: Discussionmentioning

confidence: 99%

Sickle Cell Disease (SCD) Leading to Pulmonary Arterial Hypertension (PAH) and Cholelithiasis (CL)

Chacko¹,

Jadhav²,

Ghewade³

et al. 2023

Cureus

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“…Management requires a multidisciplinary approach to target both systemic illnesses and visual defects. Hydroxyurea promotes the production of hemoglobin F, diluting the percentage of deformed red blood cells and decreasing further risk for complications [ 15 , 16 ]. In cases of neovascularization, scatter photocoagulation can be considered if the areas do not undergo auto-infarction [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

Rare Cause of Acute Loss of Vision in a Patient With Sickle Cell Trait

Bojja,

Javed,

Allena

et al. 2023

Cureus

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Sickle cell disease (SCD) is a prevalent inherited blood disorder with various ocular manifestations, including sickle cell retinopathy (SCR), characterized by retinal microcirculation impairment and ischemic complications. We present the case of a 21-year-old male with sickle cell trait who experienced a sudden, painless loss of vision in his left eye. Ophthalmologic examination revealed vitreous hemorrhage and neovascularization, indicating SCR. Initial treatment with hydroxyurea and exchange transfusions led to partial improvement. However, due to persistent vitreous hemorrhage, the patient underwent a vitrectomy. The sickle cell trait affects a large global population, and its retinopathy is a rare but severe complication. The pathogenesis and risk factors for SCR are similar to those for SCD. The diagnosis of SCR is established through fundoscopic examination and graded based on Goldberg's classification. Management involves a multidisciplinary approach targeting systemic illness and visual defects, including hydroxyurea, photocoagulation, anti-vascular endothelial growth factors, and vitrectomy. Awareness, early diagnosis, and timely intervention are essential to preventing vision-threatening complications in sickle cell trait patients with SCR.

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“…Backgroundepidemiology and pathophysiology SCD-related PH is a multi-aetiological and factorial condition and may include chronic thromboembolic disease and microvascular pulmonary occlusions, haemolytic anaemia associated with low nitric oxide and increased pro-inflammatory factors such as endogenous damage-associated molecular patterns, adenosine deaminase, purine nucleoside phosphorylase, asymmetric dimethylarginine, reactive oxygen species, NADPH, superoxide dismutases and others, left ventricular dysfunction due to anaemia-induced hypercirculation and hypoxic pulmonary vasoconstriction associated with chronic lung disease related parenchymal destruction [19,20]. According to registry data, half of patients with SCD reveal pulmonary complications and 6-10% of patients with SCD reveal PH with an equal distribution of pre-and post-capillary PH [20,21].…”

Section: Right Heart Catheterisationmentioning

confidence: 99%

Clinical–radiological–pathological correlation in pulmonary hypertension with unclear and/or multifactorial mechanisms

Lichtblau,

Mayer,

Gopalan

et al. 2023

Eur Respir Rev

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Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and/or multifactorial underlying pathologies. Management of these patients is challenging as the number of patients within these groups is often small, not all individuals with certain underlying conditions are affected by PH and patients exhibit distinct symptoms due to different underlying diseases. Studies and clinical trials in these groups are largely lacking and mostly restricted to case series and registry reports. Nonetheless, the worldwide burden of group 5 PH is estimated to be significant in terms of the prevalence of some associated diseases. Group 5 PH encompasses six subgroups, including haematological disorders (inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders), systemic disorders (sarcoidosis, pulmonary Langerhans's cell histiocytosis and neurofibromatosis type 1), metabolic disorders (glycogen storage diseases and Gaucher disease), chronic renal failure with or without haemodialysis, pulmonary tumour thrombotic microangiopathy and fibrosing mediastinitis.

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Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature

Cited by 9 publications

References 32 publications

Sickle Cell Disease (SCD) Leading to Pulmonary Arterial Hypertension (PAH) and Cholelithiasis (CL)

Sickle Cell Disease (SCD) Leading to Pulmonary Arterial Hypertension (PAH) and Cholelithiasis (CL)

Rare Cause of Acute Loss of Vision in a Patient With Sickle Cell Trait

Clinical–radiological–pathological correlation in pulmonary hypertension with unclear and/or multifactorial mechanisms

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