Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.
Arch Dermatol 1977 May
PMID:Immunoblastic lymphadenopathy with purpura and cryoglobulinemia. 30 Oct 9

Two brothers are described with ichthyosis, hepatosplenomegaly and signs of cerebellar degeneration. The ichthyosis was similar to autosomal dominant ichthyosis both clinically and biochemically. No metabolic abnormality has yet been identified to account for this previously undescribed triad of abnormalities.
Br J Dermatol 1979 May
PMID:A syndrome of ichthyosis, hepatosplenomegaly and cerebellar degeneration. 44 32

A Mexican-American boy presented at birth with an extensive eruption consisting of 0.5 to 1.0 cm hyperpigmented macules with a distinct peripheral scale involving primarily the forearms, abdomen and lower back (Fig. 1). Rare intact vesicopustules were also identified. There was an unremarkable prenatal history, and the infant was a product of a normal vaginal delivery. With the exception of the skin lesions and moderate hepatosplenomegaly, the physical examination was normal. Gram stains of the pustules showed numerous neutrophils but no bacteria. Bacterial cultures, of the skin and blood, TORCH screen (toxoplasmosis, rubella, cytomegalic virus, and herpes virus) and a VDRL were negative. On the second day of life, the patient developed several pustules with surrounding erythema consistent with erythema toxicum neonatorum. Wright-stained smears of these lesions showed abundant eosinophils. Hepatosplenomegaly resolved by the third day of life and at the time of discharge only hyperpigmented macules persisted. Follow-up visit six weeks later showed no evidence of skin lesions.
Int J Dermatol 1979 Oct
PMID:Transient neonatal pustular melanosis. 51 27

Twenty-one patients with ATL were assessed. The predominant physical findings were lymph node and bone marrow involvement, skin involvement, hepatosplenomegaly and leukemic manifestations. The predominant histopathological findings in both skin and lymph node specimens were the diffuse medium-sized cell type and the diffuse mixed cell type. Some phenotypic discrepancy was found between the neoplastic cells in the peripheral blood, lymph nodes and skin of patients with ATL with respect to CD45RA and CD45RO, and CD7, CD29, CD25 and HLA-DR. That is, the predominant neoplastic cell phenotype was the helper T-cell, which was CD3+, CD4+, CD7+, CD25+, CD45RA+ and HLA-DR+, and CD29- and CD45RO- in peripheral blood and lymph nodes, and CD3+, CD4+, CD7+, CD29+, CD45RO+ and HLA-DR+, and CD45RA- in the skin. In other words, we have described the phenotypic heterogeneity of ATL cells and demonstrated the heterogeneity of CD45R isoform expression on ATL cells in different organs--the skin, peripheral blood and lymph nodes--of the same patient.
Exp Dermatol 1992 Dec
PMID:Adult T-cell leukemia/lymphoma (ATL)--clinical, histopathological, immunological and immunohistochemical characteristics. 136 26

A 52-year-old Japanese woman with the chief complaint of marked swelling of her upper eyelids and a mass over the bulbar conjunctiva is reported. She previously noticed frequent purpura after minimal trauma, which was resolved shortly after taking some ascorbic acid. Laboratory data showed Ig-G kappa type M-protein in the serum and kappa type Bence-Jones protein in the urine by immunoelectrophoresis. Systemic examination showed mild hepatosplenomegaly, 1st degree of AV block, and a mild increase in plasma cells in the bone marrow biopsy. Histologically, the whole dermis of the eyelid skin and conjunctiva was replaced by a large quantity of amorphous, eosinophilic substances, which were diffusely positive with Direct Fast Scarlet 4BS. Immunohistochemical staining was positive for anti-amyloid P component antibody. Typical amyloid fibrils were proved by electron microscopy. She was finally diagnosed as primary systemic amyloidosis with diffuse swollen eyelids and conjunctival mass, symptoms which in primary systemic amyloidosis are very rare.
J Dermatol 1992 Feb
PMID:Primary systemic amyloidosis: a unique case complaining of diffuse eyelid swelling and conjunctival involvement. 137 22

Sexually transmitted diseases (STDs) in children may be transmitted by sexual abuse, by accidental contact, or perinatally. Although only 2% to 10% of abused children become infected, childhood syphilis, gonorrhea, condylomata acuminata, and Chlamydia trachomatis must always be considered. We reviewed data from our hospital regarding the frequency, prevalence, routes of transmission, and clinical features of these infections. Ninety-five percent of acquired syphilis in children is transmitted by sexual abuse. The perpetrator is usually someone the child knows or trusts. Of our 21 patients under 14 years of age with acquired syphilis, most were 4 to 8 years old. Girls were infected twice as often as boys. Sexual contact was confirmed in 71.4%. A chancre sore was infrequent in children; condylomata lata was the most frequent cutaneous lesion (80.9%). In the last 10 years, 102 cases of congenital syphilis were diagnosed in our hospital. The main clinical findings were bone involvement (78.7%), hepatosplenomegaly (68.8%), cutaneous lesions (50.8%), and jaundice (15.1%). Gonorrhea was detected in only nine children. Vulvovaginitis was the most common clinical manifestation. Sexual transmission was documented in three patients. Accidental contact with their infected mother occurred in two sisters. Three newborns acquired the disease during delivery. The STDs in children are a worrisome problem. Evaluation for sexual abuse should be done in all cases. Prevention and treatment of adults are the main steps to prevent these infections in children.
Pediatr Dermatol 1992 Dec
PMID:Sexually transmitted diseases in children. 149 47

A case of intravascular malignant lymphomatosis (angiotropic large cell lymphoma), T cell type was reported. The patient, a 59-year-old woman, had reddish or violaceous indurated macules scattered over the entire body surface. Neither lymphadenopathy nor hepatosplenomegaly was recognized. A chest Roentgenogram, whole body CT scan, and 67Ga-citrate scintigraphy yielded normal findings. Serum anti-HTLV-1 antibody was negative. Histopathologically, lesions showed intravascular large mononuclear cell proliferation associated with occasional fibrin thrombi formation in the dermis to subcutis. Immunohistochemically, the large mononuclear cell immuno-phenotype had a memory T cell character. Also, both lymphocyte function-associated antigen-1s, CD11a and CD18, and intercellular adhesion molecule-1 were demonstrated on the tumor cells and vascular walls in the lesions. To our knowledge, the present case is the fourth case of intravascular malignant lymphomatosis in the T cell lineage.
J Dermatol 1992 May
PMID:A case of intravascular malignant lymphomatosis (angiotropic large-cell lymphoma) presenting memory T cell phenotype and its expression of adhesion molecules. 164 50

A 2-year-old girl showed exaggerated skin reactions to mosquito bites and associated general symptoms, including a high temperature, lymphadenopathy, and hepatosplenomegaly. Peripheral blood lymphocytes contained a high percentage of CD2+, CD3-, CD4-, CD8-, CD11b+, CD16+, CD38+, CD56+, CD57-, and HLA-DR+ large granular lymphocytes that exhibited a marked natural killer cell activity. Immunohistochemically, biopsy specimens taken from the lesional skin demonstrated an infiltrate of the cells bearing the natural killer cell phenotype, indicating a role of these cells in the development of the abnormal skin reactions to mosquito bites and other systemic manifestations. Our case suggests that natural killer cell lymphocytosis may show severe hypersensitivity to mosquito bites as the most outstanding manifestation.
Arch Dermatol 1990 Mar
PMID:Severe hypersensitivity to mosquito bites associated with natural killer cell lymphocytosis. 168 90

Acne fulminans is a rare form of acne characterized by ulcerative nodules and associated with systemic complications. We report the case of a 17-year-old boy with cystic acne who experienced sudden onset of acne fulminans associated with fever, weight loss, hepatosplenomegaly, and erythema nodosum migrans. To the best of our knowledge, this is the first reported case of acne fulminans manifested by hepatomegaly and erythema nodosum migrans.
J Am Acad Dermatol 1991 May
PMID:Acne fulminans with hepatosplenomegaly and erythema nodosum migrans. 182 16

A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.
J Am Acad Dermatol 1991 Aug
PMID:Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. 183 95


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