Splenic Function in Amyloidosis

The clinical spectrum of light chain myeloma: A study of 35 patients with special reference to the occurrence of amyloidosis

No. 311 - Coil embolization of the splenic artery: long-term impact on splenic volume

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...

Autotransplantation of spleen tissue is an attempt for maintenance of splenic functions when splenectomy is indicated in children. It minimizes the risks of overwhelming postsplenectomy infection and it has been done in children with severe portal hypertension due to hepatosplenic mansonic schistosomiasis that underwent splenectomy. The purposes of this investigation were to study the morphology of the residual splenic tissue; to evaluate the residual filtration function of this splenosis; and to assess the immune response to polyvalent pneumococcal vaccine of these patients. Twenty-three children with portal hypertension from mansonic schistosomiasis who underwent splenectomy, ligature of the left gastric vein, autotransplantation of spleen tissue into an omental pouch were evaluated for residual splenic parenchyma and functions. Tc-99m sulfur colloid liver-spleen scans were used for detection of splenic nodules. The search for Howell Jolly bodies were used for assessing the filtration function and Enzyme-linked immunosorbent assay was used for measuring the relative rise in titter of specific pneumococcal antibodies. Splenosis was evident in all children; however, in two there were less than five splenic nodules in the greater omentum, which was considered insufficient. Howell-Jolly bodies were found in the peripheral blood only in these two patients with less evident splenosis. The immune response was adequate in 15 patients; it was intermediate in 4 patients and inadequate in 4 patients. Autotransplantation of spleen tissue into an omental pouch is efficient in maintaining the filtration splenic function in more than 90% of the cases and the immune response to pneumococcal vaccination in approximately 65% of the children.

Biomarkers of splenic function in infants with sickle cell anemia: baseline data from the BABY HUG Trial

Autoimmune glandular syndrome type 2 is a complex genetic condition where a triad of endocrinopathies is involved, namely, Addison’s disease, type 1 diabetes, and/or autoimmune thyroid disorder. The disease predisposes one to a variety of other autoimmune associations. Here, we report a rare presentation of a patient with autoimmune polyglandular syndrome type 2 (APS‐2) presenting with a 7‐year history of progressive splenic atrophy causing functional hyposplenism that ultimately progressed to anatomical asplenia (autosplenectomy) as demonstrated in the serial imaging. We postulate that the underlying cause of this presentation is also of autoimmune nature. Unlike APS‐1, which has been linked to hyposplenism, this is the first reported case of APS‐2 with similar splenic involvement. Splenic hypofunction can increase susceptibility to encapsulated bacterial infection, with overwhelming postsplenectomy infection (OPSI) being a significant threat. It is crucial that clinicians recognize the importance of providing guidance on vaccinations, antibiotic chemoprophylaxis, and patient education for individuals with asplenia or hyposplenism. If patients with APS can experience progressive splenic atrophy, we suggest long‐term follow‐up with splenic function assessment. It is yet unclear whether preemptive screening with pitted red cell count has any clinical impact in this group of patients.

BackgroundPersons with HIV (PWH) are at increased risk of pneumococcal disease. This study explored filtration and immune function of the spleen, investigating predictors of hyposplenism in PWH on prolonged antiretroviral therapy (ART).MethodWe developed a flow cytometry protocol to enumerate Howell-Jolly bodies (HJBs) and marginal zone B cells (MZBs), comparing results from splenectomized patients and healthy donors. We then analyzed HJBs and MZBs in a cross-sectional study to investigate spleen impairment in 96 PWH on prolonged ART.ResultsBased on results obtained from asplenic patients and healthy controls, we determined optimal cutoff values of 0.05% for HJBs and 10% for MZBs; these allow accurate differentiation between the 2 groups. HJB results were well correlated with microscopic enumeration of HJBs and pocked red blood cells, considered reference methods for spleen filtration. Among 96 HIV patients on prolonged ART, 9.4% exhibited impaired splenic filtration (HJB >0.05%), associated with a history of AIDS. Older HIV patients showed a higher frequency of HJBs, though only 1 exceeded the pathological threshold. MZBs were significantly decreased in 59.4% of HIV participants, with no correlation between MZB depletion and presence of HJBs.ConclusionsThese findings highlight that PWH exhibit depletion of splenic marginal zone B cells, and some demonstrate splenic filtration dysfunction, reflecting persistent splenic impairment despite extended viral suppression. Further research is needed to understand the long-term impact of HIV on splenic function and to develop strategies to improve clinical outcomes in this aging population.

The term functional asplenia has been used to refer to anatomic presence of the spleen without the ability to accumulate intravenously administered radiocolloid. Functional asplenia had previously been invariably associated with the presence of Howell-Jolly bodies in circulating erythrocytes. The fi

Here we report the experience at the Children's Hospital of Pittsburgh (CHP) with varicella zoster virus (VZV) in children with acute lymphoblastic leukemia (ALL). This record review was prompted by a patient with ALL who died suddenly of varicella hepatitis within 24 hours of presentation with a single skin lesion. We reviewed the medical records of children diagnosed with ALL at the CHP from January 1984 through December 1993, who subsequently developed VZV infection. Of 294 patients aged 0 to 15 years, 41 (14%) were identified as having had 42 episodes of VZV infection. Twenty patients (49%) had received prophylaxis with varicella zoster immunoglobulin (VZIG), and all 39 patients in whom the diagnosis was made premortem were treated with acyclovir. Twenty-nine of the 42 cases (70%) had disease limited to the skin. Thirteen cases (30%) had extracutaneous involvement, and five of these episodes (12% of all cases) ended in death. Risk factors for progressive varicella included age greater than 6 years and intensive immunosuppressive therapy at the time of exposure. Six of eight patients with progressive varicella, including two who died, had received VZIG. The clinical presentation in 10 of 13 patients with progressive disease and in four of five patients who died was dominated by severe abdominal and/or back pain. In seven cases, these symptoms preceded the development of skin lesions by several days, and in six patients were associated with extensive involvement of the spleen by varicella, as demonstrated histopathologically by the presence of Howell-Jolly bodies on peripheral-blood smear or radiographically. No patient with uncomplicated varicella was reported to have had premonitory pain. Recognition of these prodromes and suspicion of varicella even in the absence of skin lesions and even in children with a history of prior disease or VZIG administration should prompt early diagnostic and therapeutic measures.

Multiple myeloma is a neoplasm of plasma cells in the bone marrow. It is characterised by lytic lesions in the bones, marrow plasmacytosis and presence of M protein in serum and/or urine. Serum ?2 microglobulin is also raised and can be used for classification and prognostication of the disease. In the absence of M protein, the disease is known as non-secretory myeloma. It is proposed that raised ?2 microglobulin can be used for diagnosis and therapeutic guidance in the absence of M protein. A rare case of nonsecretory myeloma with neurocognitive impairment along with review of literature is being presented. The patient had multiple lytic lesions in bones with marked increase in plasma cells in bone marrow. M protein was not detectable in serum or urine but serum ?2 microglobulin was much elevated.Bangladesh Journal of Medical Science Vol.16(1) 2017 p.142-145

In the first part of this review, we described the physiological basis of splenic function and hypofunction. We also described the wide spectrum of diseases that can result in functional hyposplenism. In the second part of this review, we will be discussing the clinical picture, including complications, diagnostic methods, and management of hyposplenism.

The case presented in “Extensive yellowish masses in bilateral orbit and neck” describes a patient with necrobiotic xanthogranuloma (NXG) without paraproteinemia,1 which closely resembles the disease pattern we have observed in three previously reported NXG cases.2 These patients, besides the characteristic periorbital lesions, often present with thrombocytopenia, anemia, splenomegaly, and a history of splenectomy due to misdiagnosis, with this procedure usually showing limited improvement in hematological abnormalities. Additional features include liver dysfunction, cirrhosis, elevated globulin levels without paraproteinemia (all four cases lacked paraproteinemia1, 2), and positive antinuclear antibodies. Findings from peripheral blood smears often reveal marked stomatocytosis and macrothrombocytosis (Figure 1). Genetic testing identifies homozygous or compound heterozygous variants in the adenosine triphosphate–binding cassette subfamily G member 5 (ABCG5; OMIM 605459) or ABCG8 (OMIM 605460) genes, confirming a diagnosis of sitosterolemia (OMIM 210250). Family histories may be negative because of the often subclinical nature of ABCG5/G8 variants in carriers. Based on this pattern, we strongly suspect that this reported patient1 may also have sitosterolemia. Sitosterolemia is an autosomal recessive sterol storage disorder characterized by markedly elevated plasma plant sterol levels due to ABCG5/G8 variants.3 Clinically, the disorder manifests primarily as xanthomas, macrothrombocytopenia, anemia, arthralgia, and premature atherosclerosis.3 Because of the limited availability of plasma plant sterol testing, diagnostic clues can often be found on peripheral blood smears showing stomatocytosis and macrothrombocytosis. Genetic testing for ABCG5/G8 variants is essential for diagnosis. Given the patient's demise,1 we recommend genetic testing of the family members to confirm our hypothesis and guide future therapeutic interventions. Our findings suggest the existence of at least two phenotypic subtypes of NXG. The classic subtype commonly coexists with paraproteinemia, plasma cell dyscrasia, or other lymphoproliferative disorders. To date, only one classic NXG case has been reported to have undergone whole-exome sequencing, which was negative, and paraproteinemia developed during follow-up.4, 5 The second subtype is associated with ABCG5/G8 variants and features a disease pattern linked to sitosterolemia, typically without paraproteinemia or hematologic malignancy. Additional cases and longer follow-up are needed to confirm this distinction. None declared.

INTRODUCTION: Multiple Myeloma (MM) is a hematologic malignancy that most commonly presents with anemia and lytic bone lesions. MM is considered more aggressive if extramedullary involvement in either bone or solid organs is present. Review of literature reveals there are only a few documented extramedullary plasmacytomas that have occurred in the pancreas. We present a case of MM relapse manifested by a pancreatic plasmacytoma without recurrent bone marrow involvement. CASE DESCRIPTION/METHODS: A 66-year-old male was diagnosed with stage 1 MM in 2014 after a bone marrow biopsy demonstrated clonal plasma cells. He underwent radiation, chemotherapy and received an autologous stem cell transplant with high dose melphalan. His M spike has remained undetectable, and he has had stable lytic lesions seen on surveillance imaging. He was considered to be in remission. He presented for a surveillance PET/CT scan which demonstrated a hypermetabolic lesion in the head of the pancreas. A follow up MRI pancreas confirmed the presence of a well circumscribed 2.7 cm structure in the proximal pancreatic body. The differential diagnosis was fairly broad, based on imaging. He underwent an endoscopic ultrasound with fine needle aspiration. Flow cytometry demonstrated monoclonal plasma cells consistent with plasmacytoma, confirming relapse of his MM. The patient is now undergoing systemic chemotherapy and local radiation. DISCUSSION: MM presents with diffuse myelomatosis and can have associated solitary myeloma of the bone or involve other organs as extramedullary plasmacytomas. Extramedullary plasmacytomas are extremely rare, occurring in 5% of cases of MM and indicate a more aggressive form of disease. Of these 5%, most extramedullary plasmacytomas involve the submucosal lymphoid tissue of the upper respiratory tract, spleen, lymph nodes and liver. There have been only 50 cases of pancreatic plasmacytomas documented in the literature, making this a rare occurrence. Most of these cases had active bone marrow disease. Of these 50, only 5 documented cases of MM relapse manifested as a solitary pancreatic plasmacytoma. Similarly, our patient had no active bone marrow involvement at the time of diagnosis. Additionally, pancreatic plasmacytomas comprise less than 0.1% of all pancreatic masses. This case demonstrates that, although rare, an extramedullary plasmacytoma should be high on the differential if a pancreatic mass is visualized in the setting of previously treated MM.

BackgroundDuring COVID-19 pandemic, it is difficult to distinguish febrile patient infected by SARS-CoV-2 or bacterial causes. Howell-Jolly bodies are a well-known entity found in red blood cells. They are nuclear fragments, composed of deoxyribonucleic acid, commonly observed in the peripheral blood smears of hyposplenic or asplenic patients. Recently, similar inclusions often referred to as Howell-Jolly body-like inclusions (HJBLIs) have been reported in the neutrophils of patients with acquired immune deficiency syndrome (AIDS) and COVID-19 patient.AimTo explore whether HJBLIs in peripheral blood smear could differentiate between patients with confirmed SARS-CoV-2 and bacterial pneumonia.MethodsWe performed cross-sectional study using secondary data from COVID-19 database and re-evaluated peripheral blood smears to identify HJBLIs. We included confirmed COVID-19 adults age >18 years who were hospitalized in Dr. Hasan Sadikin General Hospital, Bandung, Indonesia from March 1st 2020–May 31st 2020. We also examined peripheral blood smears in patients with confirmed bacterial pneumonia as a control group. Clinical characteristics including disease severity, CURB-65 score, comorbidity, and the present of HJBLIs in peripheral blood smears were evaluated.ResultsOverall, 33 patients were included: 22 were confirmed COVID-19 and 11 were confirmed bacterial pneumonia. The median (interquartile range) age in COVID-19 and patients with bacterial pneumonia were 53 years (40–64) vs 57 years (53–71), respectively. Compared with patients with bacterial pneumonia, HJBLIs were significantly higher in COVID-19 patients [21/22 (80.8%) vs 5/11 (45.5%), p 0.001].ConclusionHowell-Jolly body-like inclusions could be a potential feature to help differentiate between COVID-19 and bacterial pneumonia.

Two unrelated patients are reported: one with isolated familial asplenia diagnosed postmortem, the other with isolated hyposplenism diagnosed after recurring invasive bacterial infections. Because both children died of fulminant septic shock, the importance of early diagnosis of splenic dysfunction is evident. Clues for an early diagnosis of congenital asplenia are recurrent invasive bacterial infections, Howell-Jolly bodies in the blood smear or a relative with congenital isolated asplenia. Although the guidelines for infection prevention in asplenism--patient education, antibiotic prophylaxis and vaccination--are well defined, controversy remains as to how to differentiate hyposplenism from functional asplenism. Based on the present observations, we define a patient as functionally asplenic--and therefore at risk for life-threatening infections-when Howell-Jolly bodies are present in the blood smear, a very small spleen is found by ultrasound, or splenic blood flow is compromised.