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27 Possible Causes for Hepatosplenomegaly, Pancytopenia, Polyclonal Hyperglobulinemia

  • Multicentric Castleman's Disease

    A 46-year-old man presented with a high-grade fever, multiple lymphadenopathies, hepatosplenomegaly and human immunodeficiency virus (HIV) seropositivity, without severe immunosuppression[] Diffuse lymph-node enlargement, splenomegaly and pancytopenia were detected. Induction with Rituximab was made because pancytopenia was present.[] hyperglobulinemia, elevated transaminases and renal disease. 2 , 5 , 7 Dispenzieri et al. 8 analyzed the clinical spectrum of Castleman’s disease in 113 patients.[]

  • Primary Myelofibrosis

    The main clinical manifestations of PMF are anemia, bleeding, hepatosplenomegaly, fatigue, and fever.[] This is then followed by pancytopenia (a reduction in the number of red and white blood cells, as well as platelets).[] Polyclonal hyperglobulinemia may be present. Patients with sporadic idiopathic or familial pulmonary hypertension have significant marrow fibrosis.[]

  • Castleman Disease

    For multicentric disease: multi-region CT bilateral hilar and mediastinal lymphadenopathy centrilobular nodules diffuse abdominal lymphadenopathy hepatosplenomegaly ascites[] There is often significant anemia which may be hemolytic, often reflecting pancytopenia or hemophagocytic syndrome (Stebbing 2009).[] The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[]

  • Angioimmunoblastic Lymphadenopathy

    AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia.[] Laboratory findings included progressive pancytopenia, hyperuricemia, and acute renal failure. Bone marrow biopsy showed a T cell lymphoid neoplasm that had ...[] In one series, other symptoms included weight loss (58%), hepatomegaly (60%), polyclonal hyperglobulinemia (65%), and generalized adenopathy (87%).[]

  • HIV Infection

    Additional symptoms commonly observed in pediatric patients comprise persistent hepatosplenomegaly, extensive molluscum contagiosum, extensive wart virus infection, and linear[] […] osteomalacia* Protease inhibitors with statins: myopathy Hematologic or oncologic Anemia of chronic disease Lymphoma, multiple myeloma Bone marrow infiltration (leading to pancytopenia[] Hyperactivation is reflected in dramatic polyclonal hyperglobulinemia, only a portion of which is directed against HIV antigens;(59) bone marrow plasmacytosis;(60) heightened[]

  • Pediatric Chronic Granulomatous Disease

    In addition, septicopyemia, thrush, and hepatosplenomegaly were also commonly observed, accounting for 23%, 23%, and 40% of the cases.[] He developed pancytopenia, hypofibrinogenemia, transaminitis, and elevated ferritin of 11 783 ng/mL.[] Polyclonal hyperglobulinemia 3. Elevated acute phase reactants such as ESR or CRP 4. Normal studies of T and B lymphocyte immunity Diagnostic test 1.[]

  • H Syndrome

    H syndrome refers to the major clinical findings of hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and occasionally[] IVIC Syndrome with mild thrombocytopenia and leukocytosis; 4) WT Syndrome involving a wide array of hematologic abnormalities including easy bruising, hypoplastic anemia, pancytopenia[] In addition, patients displayed short stature, sensorineural hearing loss, cardiac anomalies, hepatosplenomegaly, and scrotal masses.[]

  • Autosomal Dominant Autoimmune Lymphoproliferative Syndrome Type 1

    Patients with ALPS can develop a myriad of clinical manifestations including lymphadenopathy, hepatosplenomegaly, autoimmunity and increased rates of malignancy.[] […] lymphoproliferative syndrome (ALPS) is a rare, newly recognized, chronic lymphoproliferative disorder in children and is characterized by lymphadenopathy, splenomegaly, pancytopenia[] NOS ( R77.1 ) monoclonal gammopathy (of undetermined significance) ( D47.2 ) Excludes2 transplant failure and rejection ( T86 .-) D89.0 Polyclonal hypergammaglobulinemia[]

  • Congenital Intrinsic Factor Deficiency

    Anhidrotic Ectodermal Dysplasia with Immunodeficiency, FILS Facial dysmorphism, immunodeficiency, livedo, and short stature, FISH Fluorescence in situ Hybridization, HSM Hepatosplenomegaly[] Gene GIF (AR) Diagnostic Test Plasma Vitamin B12, Folate Neurological Ataxia, dystonia Non-Neurological Megaloblastic anemia, pancytopenia Treatment HydroxyCobalamin Level[] NOS (R77.1) monoclonal gammopathy (of undetermined significance) (D47.2) Excludes2: transplant failure and rejection (T86.-) D89.0 Polyclonal hypergammaglobulinemia Benign[]

  • Posthemorrhagic Anaemia of the Newborn

    Renal Oxygen sensors switch on at 40 wk of gestation. 15 16 CLINICAL FINDINGS IN ANEMIA •Pallor •Tachycardia, tachypnea •Apnea • O2 requirements •Lethargy •Poor feeding •Hepatosplenomegaly[] […] with malformations 284.1 Pancytopenia Excludes: pancytopenia (due to) (with): aplastic anemia NOS (284.9) bone marrow infiltration (284.2) constitutional red blood cell aplasia[] NOS (R77.1) monoclonal gammopathy (of undetermined significance) (D47.2) Excludes2: transplant failure and rejection (T86.-) D89.0 Polyclonal hypergammaglobulinemia Benign[]

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