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Hepatology International Feb 2018Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed... (Review)
Review
BACKGROUND
Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations.
AIM
The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO.
RESULTS
IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO.
CONCLUSION
This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.
Topics: Animals; Esophageal and Gastric Varices; Gastrointestinal Hemorrhage; Hemostasis, Endoscopic; Humans; Hypertension, Portal; Jugular Veins; Liver; Liver Cirrhosis; Models, Animal; Pancytopenia; Portal Pressure; Portal Vein; Splenomegaly; Ultrasonography, Doppler; Vascular Grafting; Venous Thrombosis; Idiopathic Noncirrhotic Portal Hypertension
PubMed: 29464506
DOI: 10.1007/s12072-018-9844-3 -
American Journal of Clinical Dermatology Jul 2022Methotrexate cutaneous ulceration is a rare methotrexate complication, and has only been described in case reports and case series.
BACKGROUND
Methotrexate cutaneous ulceration is a rare methotrexate complication, and has only been described in case reports and case series.
OBJECTIVE
To document patient characteristics, morphologic features, and mortality risk factors for methotrexate cutaneous ulceration.
METHODS
A systematic literature review of PubMed and Embase (last date 1 November 2021) was performed with data collected from case reports and case series. This study was limited to cases of cutaneous ulceration; presence of oral ulceration was collected from within these cases.
RESULTS
114 cases (men = 57.9%, mean age = 61 years) of methotrexate cutaneous ulceration met inclusion criteria. Psoriasis (69.3%), rheumatoid arthritis (18.4%), and mycosis fungoides (6.1%) were the most common indications for methotrexate use. Morphologies included erosions localized to psoriatic plaques (33.3%), epidermal necrosis/necrolysis (35.1%), localized ulceration (16.7%), and skin-fold erosions (5.3%). Methotrexate dose preceding toxicity varied greatly; median 20 mg/week, interquartile range 15-40 mg/week, range 5-150 mg/week. Most patients had risk factors for serum toxicity (baseline renal dysfunction = 37.8%, concurrent NSAID use = 28.1%, inadequate folic acid use = 89.1%). Thirty percent of cases involved mistakenly high methotrexate doses. Fourteen patients (12%) died. Absence of folic acid use (69% vs. 100%, p value < 0.001), pancytopenia (33% vs. 86%, p value < 0.001), and renal dysfunction at presentation (47% vs. 92%, p value < 0.001) were associated with increased mortality.
LIMITATIONS
Selection bias present due to abstraction from case reports and case series.
CONCLUSION
Methotrexate cutaneous ulceration is commonly preceded by dosage mistakes, absence of folic acid supplementation, and concurrent use of nephrotoxic medications. Renal impairment, pancytopenia, and absence of folic acid supplementation are key risk factors for mortality from this adverse medication reaction. Providers should regularly monitor methotrexate dosing adherence, drug-drug interactions, and perform routine laboratory evaluation. Index of suspicion for this toxicity should remain high given the varied clinical presentation and high mortality.
Topics: Drug Eruptions; Drug-Related Side Effects and Adverse Reactions; Folic Acid; Humans; Kidney Diseases; Male; Methotrexate; Middle Aged; Pancytopenia; Skin Neoplasms; Skin Ulcer
PubMed: 35486323
DOI: 10.1007/s40257-022-00692-1 -
The Lancet. Infectious Diseases Oct 2022
Topics: Exanthema; Fever; Humans; Pancytopenia
PubMed: 36152667
DOI: 10.1016/S1473-3099(22)00453-4 -
Medicina Clinica Mar 2017
Topics: Adult; Humans; Leukemia, Hairy Cell; Male; Pancytopenia; Splenomegaly
PubMed: 27345856
DOI: 10.1016/j.medcli.2016.05.011 -
Internal Medicine Journal Feb 2019Acquired aplastic anaemia is a rare, serious, immunologically mediated bone marrow failure syndrome, characterised by marrow hypoplasia of varying severity and... (Review)
Review
Acquired aplastic anaemia is a rare, serious, immunologically mediated bone marrow failure syndrome, characterised by marrow hypoplasia of varying severity and significant pancytopenia. Careful attention and investigation, including molecular testing, is required to confirm the diagnosis and exclude other mimicking conditions, such as inherited bone marrow failure syndromes. In a proportion of patients, the disease evolves to myelodysplasia or acute myeloid leukaemia and in some there is an association with paroxysmal nocturnal haemoglobinuria. The disease has a major impact on patient quality of life. Haemopoietic stem/progenitor cell transplantation for eligible patients with an available donor is the only current curative therapy. Other patients may receive immunosuppression, most commonly with anti-thymocyte globulin and cyclosporin. An initial response to immunosuppression is often encouraging, but relapse is common. Supportive care, including management of transfusion requirements and infections, is central to management. Promising new diagnostic tools and emerging therapies will likely transform approaches to this important, chronic and life-threatening condition.
Topics: Anemia, Aplastic; Antilymphocyte Serum; Blood Transfusion; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Pancytopenia; Recurrence
PubMed: 30324755
DOI: 10.1111/imj.14140 -
Indian Journal of Dermatology,... 2023
Topics: Humans; Pancytopenia; Paraproteinemias
PubMed: 35146977
DOI: 10.25259/IJDVL_425_2021 -
Expert Review of Hematology Nov 2022Copper is increasingly being recognized as a vital mineral required by both animals and humans. It plays a vital role in many metabolic processes such as cellular... (Review)
Review
INTRODUCTION
Copper is increasingly being recognized as a vital mineral required by both animals and humans. It plays a vital role in many metabolic processes such as cellular respiration, iron oxidation, and hemoglobin synthesis. Copper deficiency, which can be hereditary or acquired, can lead to a wide spectrum of disease processes such as ringed sideroblastic anemia, myelodysplasia, and pancytopenia. Timely identification and management of copper deficiency is necessary to prevent irreversible complications.
AREAS COVERED
Our study focuses on prevalence, etiology, pathophysiology, complications, and treatment of copper deficiency.
EXPERT OPINION
Copper deficiency is frequently underrecognized as the cause of anemia, neutropenia, and bone marrow dysplasia. As it is potentially treatable, it should always be kept in the differentials when patients present with neurological and hematological abnormalities.
Topics: Animals; Humans; Pancytopenia; Copper; Anemia; Hematologic Diseases; Neutropenia; Myelodysplastic Syndromes
PubMed: 36314081
DOI: 10.1080/17474086.2022.2142113 -
The National Medical Journal of India 2023Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the...
Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the diversity of differential diagnoses. Hepatosplenic T-cell lymphoma is a rare and aggressive type of non-Hodgkin lymphoma that can present with massive hepatosplenomegaly, pancytopenia and prolonged fever. Most of the patients are young men and the majority are associated with chronic immunosuppression. We report a 40-year-old immunocompetent woman with prolonged fever and pancytopenia due to hepatosplenic T-cell lymphoma.
Topics: Humans; Pancytopenia; Adult; Female; Splenomegaly; Hepatomegaly; Fever; Lymphoma, T-Cell; Splenic Neoplasms; Liver Neoplasms; Diagnosis, Differential
PubMed: 38692598
DOI: 10.25259/NMJI_207_20 -
Clinical Chemistry Aug 2019
Topics: Female; Humans; Liver Function Tests; Pancytopenia; Peripheral Nervous System Diseases
PubMed: 31358502
DOI: 10.1373/clinchem.2019.302927 -
The Journal of the Royal College of... Dec 2018Hyperparathyroidism may be a precipitating factor to the development of myelofibrosis; however, this is extremely rare with only a few documented case reports of...
Hyperparathyroidism may be a precipitating factor to the development of myelofibrosis; however, this is extremely rare with only a few documented case reports of myelofibrosis caused by secondary hyperparathyroidism. We describe a case of a 24-year-old female who had a failed live donor renal transplant and secondary hyperparathyroidism. While on haemodialysis she became increasingly pancytopenic despite erythropoietin injections and adequate iron, vitamin B12 and folate replacement. Her secondary hyperparathyroidism evolved to tertiary hyperparathyroidism despite vitamin D supplementation and phosphate binders. In order to determine the cause of her pancytopenia, a bone marrow biopsy was performed that confirmed myelofibrosis due to her secondary hyperparathyroidism. Following a successful parathyroidectomy in a tertiary hospital, her pancytopenia resolved and she is now awaiting a second transplant.
Topics: Bone Marrow; Female; Fibrosis; Humans; Hyperparathyroidism, Secondary; Kidney Failure, Chronic; Kidney Transplantation; Pancytopenia; Renal Dialysis; Young Adult
PubMed: 30488885
DOI: 10.4997/JRCPE.2018.406