- Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH). [Case Reports]
- CRCase Rep Hematol 2019; 2019:3146965
- Autosplenectomy (AS) is a known complication of diseases such as sickle cell anemia, celiac disease, and inflammatory bowel disease. We report the first known case of AS due to paroxysmal nocturnal h…
Autosplenectomy (AS) is a known complication of diseases such as sickle cell anemia, celiac disease, and inflammatory bowel disease. We report the first known case of AS due to paroxysmal nocturnal hemoglobinuria (PNH). A 24-year-old Caucasian male had evidence of hemolytic anemia at the age of 14 and was diagnosed with PNH at the age of 16. He had recurrent episodes of sepsis due to dialysis line infections from poor hygiene, and blood cultures had been positive for multiple organisms including Staphylococcus aureus, Enterococcus faecalis, and Streptococcus pneumoniae. The patient's peripheral blood smears since the age of 14 years demonstrated Howell-Jolly bodies in conjunction with thrombocytopenia and hemolytic anemia, but abdominal ultrasonography reported a normal appearing spleen. The patient presented with septicemia two years after starting eculizumab, and his peripheral blood smear showed extensive Howell-Jolly bodies, Pappenheimer bodies, acanthocytes, and target cells. Splenic ultrasonography demonstrated an atrophic spleen with multifocal scarring, and absent splenic uptake of liver-spleen scintigraphy, consistent with AS. Clinicians should remain vigilant of the potential sequelae of PNH and consider the possibility of the development of AS.
- Morbidity pattern and interferon gamma level in sickle cell anemia patients with autosplenectomy. [Journal Article]
- NJNiger J Clin Pract 2018; 21(12):1615-1621
- CONCLUSIONS: This study showed a higher prevalence of infection and bone pain crisis among SCA patients with autosplenectomy than in SCA patients without autosplenectomy. It also showed comparable level of IFN-γ in the 2 groups of patients. Patients with autosplenectomy may benefit from early institution of drugs such as hydroxyurea to improve quality of life.
- Sonographic Evaluation of Some Abdominal Organs in Sickle Cell Disease Patients in a Tertiary Health Institution in Northeastern Nigeria. [Journal Article]
- JMJ Med Ultrasound 2018 Jan-Mar; 26(1):31-36
- CONCLUSIONS: Abdominal sonography in SCD patients revealed varied remarkable changes in the size, echotexture, intraluminal deposits and wall thickness in the liver, gallbladder, kidneys, and spleen.
- Inflammation in sickle cell disease. [Journal Article]
- CHClin Hemorheol Microcirc 2018; 68(2-3):263-299
- The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processe…
The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processes that ultimately lead to vaso-occlusion. In addition to their role in the initiation of the acute painful vaso-occlusive episodes that are characteristic of SCD, inflammatory processes are also key components of many of the complications of the disease including autosplenectomy, acute chest syndrome, pulmonary hypertension, leg ulcers, nephropathy and stroke. We, herein, discuss the events that trigger inflammation in the disease, as well as the mechanisms, inflammatory molecules and cells that propagate these inflammatory processes. Given the central role that inflammation plays in SCD pathophysiology, many of the therapeutic approaches currently under pre-clinical and clinical development for the treatment of SCD endeavor to counter aspects or specific molecules of these inflammatory processes and it is possible that, in the future, we will see anti-inflammatory drugs being used either together with, or in place of, hydroxyurea in those SCD patients for whom hematopoietic stem cell transplants and evolving gene therapies are not a viable option.
- A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude. [Case Reports]
- WEWilderness Environ Med 2018; 29(1):85-89
- A 24-year-old man presented with acute abdominal pain upon ascent to moderate altitude (3500 m). An immediate evaluation revealed a splenic infarct, and he was evacuated to sea level. Upon recovery, …
A 24-year-old man presented with acute abdominal pain upon ascent to moderate altitude (3500 m). An immediate evaluation revealed a splenic infarct, and he was evacuated to sea level. Upon recovery, he was sent back to 3500 m without detailed etiological evaluation, whereupon he experienced recurrent episodes of left-side subcostal pain. Imaging suggested autosplenectomy, and workup revealed a negative thrombophilia profile but was positive for sickle cell trait (SCT). Individuals with SCT can be asymptomatic until exposure to severe hypoxia, upon which they can manifest clinically as sickle cell syndrome. We discuss the rare presentation of autosplenectomy in a patient with previously undiagnosed SCT on exposure to high altitude.
- Diagnostic challenges of prolonged post-treatment clearance of Plasmodium nucleic acids in a pre-transplant autosplenectomized patient with sickle cell disease. [Case Reports]
- MJMalar J 2018 01 10; 17(1):23
- CONCLUSIONS: While molecular testing can provide sensitive Plasmodium nucleic acid detection, the persistence of Plasmodium nucleic acids following adequate treatment in functionally asplenic patients can lead to a diagnostic dilemma. In such patients, clinical response and peripheral blood smears should guide patient management following treatment. Nonetheless, in pre-transplant patients at high-risk for pre-existing Plasmodium infections, highly sensitive molecular assays can be useful to rule out infection prior to transplantation.
- Is thrombocytosis always an indicator of autosplenectomy in patients with systemic lupus erythematosus? [Journal Article]
- RIRheumatol Int 2018; 38(2):239-247
- In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type an…
In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis". Peripheral smear results of patients with thrombocytosis were analyzed, and spleen imaging was performed for autosplenectomy/hyposplenism to patients with persistent thrombocytosis. A total of 205 patients with SLE were included in the study [196 (95.6%) female, mean age 41.5 years]. Out of 12 patients (5.9%) with thrombocytosis, 9 (4.3%) had transient thrombocytosis and 3 patients (1.4%) had persistent thrombocytosis. Of those with transient thrombocytosis, 5 were associated with iron deficiency anemia (IDA), 2 to polyarthritis, and the remaining 2 to digital ischemia and/or cutaneous vasculitis. Of three patients with persistent thrombocytosis, one was identified to have had splenectomy due to resistant immune thrombocytopenic purpura, and the other two (0.9%) patients had autosplenectomy. The only independent risk factor for the development of thrombocytosis was the presence of cutaneous vasculitis (OR 10.79 (95% CI 2.14-54.47), p = 0.0004). During the course of SLE, frequency of thrombocytosis is similar to that of the general population and the most common cause is reactive thrombocytosis. If the thrombocytosis was persistent, rheumatologist must consider that the patient may have autosplenectomy/asplenia/hyposplenism.
- A case of autosplenectomy associated with T-cell checkpoint inhibitor treatment. [Case Reports]
- BCBMJ Case Rep 2017 Oct 19; 2017
- Invasive Pneumococcal Disease in Patients With Sickle Cell Disease. [Journal Article]
- JPJ Pediatr Hematol Oncol 2017; 39(5):341-344
- CONCLUSIONS: Breakthrough cases of IPD may involve nonvaccine isolates, and seem to occur after 5 years of age when oral penicillin prophylaxis has been terminated.
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- The case of the mysterious vanishing spleen: autosplenectomy complicating pneumococcal sepsis. [Case Reports]
- BCBMJ Case Rep 2017 May 04; 2017
- A 57-year-old previously healthy fisherman was admitted in fulminant pneumococcal septic shock, with disseminated intravascular coagulation, requiring aggressive management including bilateral below-…
A 57-year-old previously healthy fisherman was admitted in fulminant pneumococcal septic shock, with disseminated intravascular coagulation, requiring aggressive management including bilateral below-knee amputations for ischaemic necrosis. He began to recover and was discharged for rehabilitation, however during his convalescence was found to be hypercalcaemic. No malignancy was found on CT scan, but it was noted that his spleen was absent, replaced by a 4 cm smooth-walled, fluid-filled lesion. This was unexpected as an ultrasound in intensive care 10 weeks previously had demonstrated a normal spleen. Functional hyposplenism was confirmed on a peripheral blood film with evidence of target cells, spherocytes and Howell-Jolly bodies. A diagnosis of autosplenectomy complicating pneumococcal sepsis was therefore made, of which there is just one case previously reported. The patient continues to recover well and was discharged on penicillin prophylaxis after receiving vaccinations for hyposplenism.