2002 Characteristic microorganisms are readily seen within these cells by using electron microscopy (see Fig. Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of SciencesDisclosure: Nothing to disclose. [Medline]. [18, 20] Splenectomy is therapeutic in individuals with severe pancytopenia due to splenomegaly. Joerg Mayer Dr. [Medline]. 2011 Nov. 155 (3):308-17. Interestingly, B cells in each of these may respond differently to immunosuppressive therapy [104]. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA2MjA4LXdvcmt1cA==. American Association for the Advancement of Science, American Clinical and Climatological Association, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada, American Association for Cancer Education, American College of Clinical Pharmacology, American Society for Clinical Investigation, Association of Military Surgeons of the US, Society for Experimental Biology and Medicine, Southern Society for Clinical Investigation, American Association for the Surgery of Trauma, Eastern Association for the Surgery of Trauma, International Trauma Anesthesia and Critical Care Society, Society for the Advancement of Blood Management. The erythrocyte sedimentation rate is usually increased; anemia and microscopic hematuria are also found. For spleen scanning, erythrocytes are labeled with chromium-51 (51Cr) , mercury-197 (197Hg), rubidium-81 (81Rb), or technetium-99m (99mTc), and the cells are altered by treatment with heat, antibody, chemicals, or metal ions so that the spleen will sequester them after infusion. Did the Wise Men Notice the Babinski Sign in the Christ Child? Diseases & Conditions, encoded search term (Splenomegaly) and Splenomegaly, Fast Five Quiz: Prognosis of Primary Myelofibrosis, Chronic Splenomegaly in a 10-Year-Old Boy, My Strangest Case: An Infant With Lumps in Her Belly, HDL Mimetic Restores Kidney Function, Vision in Rare Disorder, EMA Panel Endorses Approval of Five New Cancer Drugs, A 70-Year-Old Man With Splenomegaly: Osmosis USMLE Study Question, More Evidence for Lower Risk With Certain Blood Groups, Baby Gets Cancer From Mother During Birth: First Report. In these patients the catabolic destruction of IgE is slowed. Stephen L. Hoffman, ... Nicholas J. med.vet., MSc, Dipl. In imaging studies, a craniocaudal measurement of 11-13 cm is frequently used as the upper limit of normal splenic size. Anemia and some degree of pancytopenia are usually evident, but in many cases malarial parasites cannot be found in peripheral blood smears. Splenoportographic findings can help to identify the cause of idiopathic splenomegaly, especially in children. It seems to be, however, that there are profound abnormalities in homeostasis in various B-cell compartments in SLE. Neetu Radhakrishnan, MD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, American Society of HematologyDisclosure: Nothing to disclose. Butler JR, Eckert GJ, Zyromski NJ, Leonardi MJ, Lillemoe KD, Howard TJ. [Medline]. 2012 Mar. Bezerra AS, D''Ippolito G, Faintuch S, Szejnfeld J, Ahmed M. Determination of splenomegaly by CT: is there a place for a single measurement?. 2017 Feb. 53:47-52. Continuous IL-6 stimulation in mice produces hypergammaglobulinemia and mimics the chronic B cell hyperplasia of Castleman's disease, or giant lymph node hyperplasia. Cochrane Database Syst Rev. ACZM, Dipl. Kawanaka H, Akahoshi T, Kinjo N, et al. 224(1):19-28. Anticancer Res. Flowers JL, Lefor AT, Steers J, et al. Clinical Case, You are being redirected to 19(3):258-62. 206208-overview Gina M Matacia-Murphy, MD is a member of the following medical societies: American Society of Clinical OncologyDisclosure: Nothing to disclose. History and physical examination, laboratory studies, and CT scanning can help clinicians to determine the etiology of splenomegaly in greater than 90% of cases. Hyper-IgD syndrome has also been described. Data on the autocrine production of cytokines by B cells that act as signals to themselves and to T lymphocytes, a recently described role in promoting the spontaneous activation of T cells [100], as well as the expression by them of lymphotoxin a/b, which is essential for the differentiation of follicular dendritic cells [101], indicate that B cells may have more active participation in the immune dysregulation of SLE than previously thought [102]. In general, the spleen can be considered enlarged if its craniocaudal length is more than 10 cm on conventional CT scans. Blood cell conditions in which too few or too many of certain types of blood cells are produced, such as leukopenia, leukocytosis, thrombocytopenia, thrombocytosis, pancytopenia and polycythemia; Cancers of the blood or bone marrow, including leukemias, lymphomas and multiple myeloma Splenic biopsy may be performed in specialized institutions. From: Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases (Eighth Edition), 2015, Philip R. Greipp, in Encyclopedia of Immunology (Second Edition), 1998. Ann Surg. [Full Text]. 135(3):385-8. Mosquera-Klinger G, de la Serna Higuera C, Bazaga S, García-Alonso FJ, Sánchez Ocaña R, Antolín Melero B, et al. Point-of-care ultrasonography significantly improved the sensitivity of physical examination alone in diagnosing splenomegaly in a prospective study of 39 adult hospitalized patients. Vasculitis Cases of vasculitis resulting in severe complications and death have been reported in patients receiving Propylthiouracil therapy. Note that the massively enlarged spleen is readily visible, with minimal retraction in the left upper quadrant. Splenectomy for hematologic disease. Several mechanisms might be responsible for the increased B cell activation, including the gut breach. Splenectomy is typically performed laparoscopically; even supramassive spleens can be removed by laparoscopic surgery with minimal morbidity. Splenic biopsy may be performed in specialized institutions. Copyright © 2021 Elsevier B.V. or its licensors or contributors. Olson AP, Trappey B, Wagner M, Newman M, Nixon LJ, Schnobrich D. Point-of-care ultrasonography improves the diagnosis of splenomegaly in hospitalized patients. Kimmel et al. An update. Patients have characteristic vascular purpura of the lower extremities. 1963 to 1995: 449 patients. Intraoperative photograph of a laparoscopic splenectomy being taken down using the hanging-pedicle technique. Scott D. Cohen, ... Paul L. Kimmel, in Chronic Renal Disease, 2015, Polyclonal hypergammaglobulinemia frequently occurs in the setting of HIV infection.16 This predisposes to the development of circulating immune complexes. 2007 Jan 5. The evaluation of isolated neutropenia, anemia, and … FPnotebook.com is a rapid access, point-of-care medical reference for primary care and emergency clinicians. Persons with HMS who are living in endemic areas should receive antimalarial chemoprophylaxis; the results are usually good. 1998 Jun. [Full Text]. Endoscopic ultrasound-guided fine-needle aspiration for splenomegaly and focal splenic lesion: is it safe, effective and necessary?. In AIDS, there is a decreased CD4/CD8 T cell ratio, yet the level of polyclonal Ig is paradoxically increased. O'Reilly RA. A multicentre observational study for early diagnosis of Gaucher disease in patients with Splenomegaly and/or Thrombocytopenia. Microscopically, there is a subacute and chronic inflammatory infiltrate. The development of linear IgG deposits at the dermo-epidermal junction appears to be a good indicator for the existence of autoantibodies and immune complex glomerulonephritis (van Elven et al., 1981). Both κ and λ light chains are represented. All pulses are full and equal throughout. 2009 May 20. Severe bleeding is a frequent complication that limits the usefulness of this procedure. [Medline]. This immunologic process stimulates reticuloendothelial hyperplasia and clearance activity and eventually produces splenomegaly. [Medline]. A monoclonal gammopathy or B cell lymphoma may develop in patients with Castleman's disease or Sjögrens syndrome. Episode 88 - Student Virtual Morning Report - July 5, 2020 - Fatigue Increase in serum β2-microglobulin (the light chain of the class I major histocompatibility complex) accompanies the polyclonal hypergammaglobulinemia seen in lupus and other autoimmune disorders such as Sjögren's syndrome. Rarely, no underlying cause is found, and the increase is attributed to an unknown antigen exposure or defective regulation of B cell proliferation and differentiation. Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen: prepared on behalf of the British Committee for Standards in Haematology by a working party of the Haemato-Oncology task force. Massive splenomegaly does not preclude splenectomy through a minimally invasive approach. [Medline]. Some residents of malaria-endemic areas of tropical Africa and Asia exhibit an abnormal immunologic response to repeated infection that is characterized by massive splenomegaly, hepatomegaly, marked elevations in serum titers of IgM and malarial antibody, hepatic sinusoidal lymphocytosis, and (in Africa) peripheral B-cell lymphocytosis. An estimated 20 percent of American children will have anemia at some point in their childhood.1 Anemia is defined as a hemoglobin (Hgb) concentration or red … West J Med. 2002 Sep-Oct. 2 (5):440-3. In hypersplenism, the platelet count is usually 50,000-150,000/µL. A CT scan remains the most useful preoperative investigation to measure splenic volume; to exclude lymph nodes at the splenic hilum; and to detect accessory spleens, splenic abscesses, and perisplenitis. Splenectomy in hematologic disorders. Subhasis RC, Rajiv C, Kumar SA, Kumar AV, Kumar PA. Surgical treatment of massive splenomegaly and severe hypersplenism secondary to extrahepatic portal venous obstruction in children. A splenic length of greater than 14 cm is considered enlarged on liver-spleen scan. Whether these reflect passively the polyclonal B-cell activation itself, are antigen driven, or are the result of a somatic hypermutation of germline gene-encoded natural autoantibodies is difficult to tell at this time. Chin Med J (Engl). Hyper-gammaglobulinemia is a hallmark of HIV infection along with polyclonal B cell activation. Crit Ultrasound J. A massive spleen that was removed from an elderly woman with lymphoma. Among these are non-Hodgkins and Hodgkins lymphoma and acute lymphocytic and myelogenous leukemia. The gamma globulin increase in Sjögrens syndrome is characteristically associated with very high levels of rheumatoid factor (IgM anti-IgG immunoglobulin). 9:CD003425. [Medline]. Bone marrow cytology or histology in dogs with chronic CME (myelosuppression) will have a marked reduction in hematopoietic tissue. 16(6):749-57. 2014 Mar 27. The composition of peripheral blood B cells in HIV patients is also different in which some populations that normally reside in the bone marrow, including immature B cells and plasmablasts, begin to circulate in blood [38]. Hyper-IgM syndrome is described in another article in the encyclopedia. Whether this role is primary or results from the effects of T cells, monocytes, dendritic, or NK cells is yet unknown. 2015 Feb 17. In most cases of pancytopenia, referral to a hematologist will be important for purposes of diagnosis and/or management. Autoimmune pancreatitis is characterized by hypergammaglobulinemia, histologic evidence of lymphoplasmacytic inflammation and fibrosis, and a favorable response to glucocorticoid treatment. 1979 Feb. 66(2):311-20. Laparoscopic splenectomy in patients with hematologic diseases. Br J Haematol. These findings suggest that autoimmune mechanisms are involved in the pathogenesis of this disease. There is no underlying infectious or inflammatory condition, and there is no evidence of an autoimmune disease or lymphatic neoplasm. 2009 Sep. 24(9):1554-8. The mechanisms for those blood count abnormalities are as follows: The underlying histologic anatomy of the spleen largely determines its characteristic appearance on abdominal computed tomography (CT) scans. Physical examination findings are normal except for mild splenomegaly and signs of early gangrene in the right second toe. [Full Text]. Am J Med. 2015 Dec. 7 (1):13. Thrombocytopenia -  Increased splenic platelet pooling is the primary cause of the thrombocytopenia of hypersplenism. One example is the acquired immune deficiency syndrome (AIDS). 2009 Jun. Splenomegaly and hepatomegaly are found in slightly more than 50% of patients. Findings that indicate radiologic distinction between benign and malignant lesions are inconsistent and cannot be relied on to establish or refute a diagnosis of malignancy. Patients with natural killer (NK) cell/large granular lymphocyte (LGL) syndrome have a polyclonal hypergammaglobulinemia. Am J Surg. [Medline]. A spleen scan is also useful for detecting space-occupying lesions in the splenic substance, evaluating loss of splenic functions, assessing for the absence of a spleen, or determining the presence of an accessory spleen. Under the influence of cytokines such as interleukin 6 (IL-6), these B cells differentiate into antibody-producing plasma cells. 2011 Dec. 13(12):839-45. These polyclonal plasma cells increase in number in the bone marrow, gut, lymph nodes, spleen and liver. All material on this website is protected by copyright, Copyright © 1994-2021 by WebMD LLC. 2007. [Medline]. 17. [Medline]. However, several issues remain to be clarified: the target antigen recognized by IgG4 autoantibody and the role of IgG4 elevation, details of extrapancreatic lesions with respect to systemic disease, therapeutic strategies, and long-term outcome with respect to the pancreatic structure and function, and predisposition to malignancy. The anti-Ro/SSA and anti-La/SSB B-cell responses appear linked, with patients who have anti-La/SSB antibodies present in their sera almost always having detectable anti-Ro/SSA antibodies as well. Abdominal ultrasonography (US) shows characteristic sonolucent swelling—“sausage-like” appearance, and dilatation of the common bile duct. Ginzel AW, Kransdorf MJ, Peterson JJ, Garner HW, Murphey MD. GVHR is invariably associated with hypergammaglobulinemia, which can be easily determined by solid-phase enzyme-linked immunosorbent assay (ELISA). Surg Laparosc Endosc Percutan Tech. Splenic biopsy. 2011 Nov 20. However, because of wide variations in shape, no consistent correlation has been recognized between the spleen's length and its overall volume, as has been determined for other organs (eg, kidney). 1988 May. Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 53(3):430-4. If we return to the mice models, we find that anti-dsDNA B cells in nonautoimmune mice exit from the bone marrow to populate the spleen where they locate at the T–B interface of the splenic follicle where they have an increased in vivo turnover rate [98]. Lavrenkov K, Krepel-Volsky S, Levi I, Ariad S. Low dose palliative radiotherapy for splenomegaly in hematologic disorders. 313 (7):695-706. Marked clubbing of the fingers and hypergammaglobulinemia are frequently present. When referring to an enlarged spleen as hypertrophied, the underlying cause may be hypertrophy or hyperplasia of individual cells. Another characteristic feature of this disease is high serum IgG4 concentrations, which are closely associated with disease activity. Still another group of patients may have hyper-IgG syndrome. In systemic lupus erythematosus, the increase in polyclonal immunoglobulin is usually modest but many different autoantibodies may be present, such as antinuclear antibody, rheumatoid factor, cardiolipin antibodies and antibodies against red blood cells, neutrophils and platelets. Arakawa Y, Shimada M, Utsunomiya T, Imura S, Morine Y, Ikemoto T, et al. Complete blood cell count (CBC) with differential, Evaluation of peripheral blood smear for RBC morphology and signs of myeloproliferative disorders or underlying bone marrow disorders, Prothrombin time with international normalized ratio (INR) and activated partial thromboplastin time (aPTT). Rarely, the polyclonal hypergammaglobulinemia may be restricted to one or more subclasses of immunoglobulin (Ig). This disease has been frequently misdiagnosed as pancreatic cancer, leading to unnecessary surgery. The ever-changing indications. Leukopenia is closely related to neutropenia. Xu WL, Li SL, Wang Y, Shi BJ, Li M, Li YC, et al. The fate of autoreactive B cells within a monoclonal B-cell repertoire may be different from that in a polyclonal one [97]. Share cases and questions with Physicians on Medscape consult. Two forms exist, including the "sago spleen," in which amyloid deposits are limited to follicles, and the "lardaceous spleen," in which amyloid is deposited in the walls of the splenic sinusoids. Inferiorly, it extends into the pelvis. Clin Med (Lond). 120(1):41-5. Wang KX, Hu SY, Zhang GY, Chen B, Zhang HF. Episode 91 - Virtual Morning Report - July 10, 2020 - Night sweats & Splenomegaly. Polyclonal hypergammaglobulinemia is usually due to an underlying infectious, inflammatory or neoplastic disorder such as a carcinoma. B and T lymphocytes have a decreased response to mitogen. Chin Med J (Engl). [Full Text]. The disease is rare, being only 1% to 2% of all leukemias. Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook, Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology, David Coffman, MD Fellow, Department of Surgery, Division of Trauma and Critical Care, Yale University School of Medicine, Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine, Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group, Disclosure: No financial interests None None, Emmanuel N Dessypris, MD Professor of Medicine, Medical College of Virginia; Chief, Medical Service, Hunter Holmes McGuire Department of Veterans Affairs Medical Center, Emmanuel N Dessypris, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society of Hematology, New York Academy of Sciences, Society for Experimental Biology and Medicine, and Southern Society for Clinical Investigation, David J Draper, MD Fellow, Department of Hematology/Oncology, The University Hospital, University of Cincinnati College of Medicine, Lewis J Kaplan, MD, FACS, FCCM, FCCP Director, SICU and Surgical Critical Care Fellowship, Associate Professor, Department of Surgery, Section of Trauma, Surgical Critical Care, and Surgical Emergencies, Yale University School of Medicine, Lewis J Kaplan, MD, FACS, FCCM, FCCP is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Surgeons, Association for Academic Surgery, Association for Surgical Education, Connecticut State Medical Society, Eastern Association for the Surgery of Trauma, International Trauma Anesthesia and Critical Care Society, Society for the Advancement of Blood Management, Society of Critical Care Medicine, and Surgical Infection Society, Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference.
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