Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe a case of stage IV-S neuroblastoma in a boy forty days old with impressive enlargement of the liver whose left lobe was initially mistaken for splenomegaly. Regression was obtained without active treatment. Factors affecting the prognosis and treatment are briefly discussed. Is reported the association with persistent elevation of serum acid phosphatase.
 ...
PMID:[Case report of a stage IV S neuroblastoma regressing without any aggressive treatment]. 664 85

From 1972 to 1981 10 patients with hairy cell leukaemia were observed in the Medical University Clinic Cologne, this represents 1.8% of all leukaemias. Typical clinical signs are splenomegaly, no or only slightly enlarged lymph nodes and a moderate hepatomegaly. Almost in all cases an anaemia, thrombocytopenia and neutropenia with lymphocytosis was found, mostly combined as pancytopenia. The pathognomonic tartrate resistant acid phosphatase was found in the hairy cells to a differing amount besides a fibrosis and a lymphatic infiltration of the bone marrow. A normalization of the anaemia, the thrombocytopenia and the neutropenia was reached by splenectomy, but the increased susceptibility to infections could not be affected significantly.
 ...
PMID:[Clinical aspects of hairy cell leukemia and its modification by splenectomy]. 676 22

The physician who diagnoses Gaucher's disease should take advantage of the noninvasive method that analyzes WBCs for residual beta-glucocerebrosidase and beta-glucosidase activities. When this method is carried out in conjunction with the measurement of serum acid phosphatase levels, a bone marrow examination may be unnecessary. With this method, we studied an adult who had mild splenomegaly and abdominal pain. When bone marrow was finally obtained subsequent to diagnosis by the enzymatic analysis, the deposits that are specifically formed in Gaucher's disease were easily demonstrated by electron microscopy. We believe that these methods are more specific for the diagnosis of Gaucher's disease than is the light microscopic finding of bone marrow cells that have abundant and striated cytoplasm.
 ...
PMID:Gaucher's disease. I. Modern enzymatic and anatomic methods of diagnosis. 677 79

In an attempt to establish a possible correlation between clinical course and initial characteristics of the disease, 88 children with ALL (diagnosed between 1970-1978) were studied. Basis for comparison was whether relapses (medullary or extramedullary) occurred within 36 months. Twenty parameters, including history data, physical exploration, laboratory findings and early response to induction treatment, were evaluated. Statistical analysis showed a significant influence of the following factors: age less than 1 year, organomegalies (hepato or splenomegaly larger than 5 cm B.C.M.), adenomegalies (more than 3 cm in diameter), mediastinal mass, initial CNS infiltration, E-rosette forming blast-cells, acid phosphatase positivity and a good hematological response after 2nd week of treatment. Sex appeared as a prognostic factor after 36 months. Three group of patients could be distinguished according to risk factors: A) Patients without risk factors; 70.45% in continuous remission (CR) after 36 months. B) Patients with 1-2: 43.47% in CR at 36 months. C) Three or more: None attained 36 months in continuous CR. Differences are significant (p less than 0.05).
 ...
PMID:[Evaluation of initial risk factors in acute lymphoblastic leukemias in children]. 696 35

Hairy-cell leukemia is characterized clinically in splenomegaly and pancytopenia and pathologically by the proliferation in hematopoietic tissue of cells containing the tartrate-resistant isozyme 5 of acid phosphatase. We have described a patient with a T-lymphocyte variant of this disease. A permanent cell line obtained from the spleen of this patient has the biological and enzymatic characteristics of the fresh leukemic cells. We have used this line to study the surface morphology, ultrastructure, and ultrastructural localization of acid phosphatase in defined T-lymphoid hairy cells. The surface of the cells of the permanent line was smooth but many hair-like projections appeared after exposure to phytohemagglutinin (PHA). There was little acid phosphatase reaction produce visualized when beta-glycerophosphate was used as a substrate. With sodium haphthol AS-BI phosphoric acid heavy deposits were seen in the perinuclear membrane, mitochondria, and rough endoplasmic reticulum. Exposure to PHA and pokeweed mitogen resulted in increased reaction product, suggesting increased enzyme synthesis. Tartrate-resistant acid phosphatase was localized in the same organelles.
 ...
PMID:Ultrastruct and tartrate-resistant acid phosphatase localization in a T-cell hairy-cell leukemia cell line. 696 46

A case of acute lymphoblastic leukemia with a giant splenomegaly in an 8-year-old boy was investigated by immunological, cytochemical and electronmicroscopical techniques. Bone marrow and peripheral blood were largely replaced by large blast cells with a nonconvoluted nucleus. Cytochemically, most of the blast cells showed strong focal acid phosphatase activity. In the surface marker studies, these blast cells formed EAC rosettes but not E rosettes, while they showed positive reactivity with anti-T lymphocyte serum but not with anti-B lymphocyte serum. This membrane phenotype E-, C3R+, T+, B- suggested that leukemic blast cells in this patient presumably originated from precursor T cells.
 ...
PMID:Precursor T cell leukemia--immunological, cytochemical and morphological studies. 697 49

Monocytoid B cell lymphoma (MBCL) is a newly recognized low-grade lymphoma. The malignant cells of MBCL seems to be the counterpart of reactive monocytoid B cells. The MBCL cells show a resemblance to the cells of hairy cell leukemia (HCL). The cell of MBCL exhibits immunoglobulin light chain restriction and markers of terminal BG cell differentiation. On the other hand, the HCL lack expression of CD 25 and are tartrate resistant, acid phosphatase-negative. The MBCL has prediction for elderly females. At the time of diagnosis, it is usually present in stage I or II. It may be difficult to differentiate the clinical and morphological expression of MBCL from that of any other indolent lymphoproliferative diseases, especially HCL and small lymphocytic lymphoma. Unlike other low grade lymphomas in MBCL, however, splenomegaly and bone marrow involvement are uncommon, while leukemic conversion is very rare. The MBCL transformation or association to high grade lymphomas is very unusual. In this case, it isn't clear if MBCL undergoes progression to higher grade lymphomas or if there is a "composite lymphoma" at the onset, in any case the prognosis is worse. In our patient, the involvement seemed to be only at the spleen and caeliac nodes, there was not bone marrow involvement. The fast evolution suggests that the MBCL underwent a transformation to a higher grade lymphoma. It is a common behavior in the low grade lymphoma.
 ...
PMID:[Monocytoid lymphoma evolving into large-cell lymphoma]. 817 84

An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes. The lesions resolved following antifungal therapy, but persisting pancytopenia and splenomegaly necessitated further hematological evaluation. A diagnosis of HCL was suspected based on morphologically characteristic hairy cells in the peripheral blood that contained tartrate resistant acid phosphatase. A bone marrow biopsy specimen had a normocellular marrow with an increase in interstitial lymphoid cells that stained with L26, MB2, and LN2 antibodies. On flow cytometry these cells were positive for the leukocyte common antigen, B cell markers, and the CD11c antigen confirming the diagnosis of HCL. We believe that this is the first report of sporotrichosis infection as a presenting manifestation of HCL.
 ...
PMID:Sporotrichosis as a presenting manifestation of hairy cell leukemia. 817 80

Forty Japanese patients with hairy cell leukemia (HCL) were reviewed. Nine cases were diagnosed as typical HCL, and two cases had the features of HCL variant (prolymphocytic variant). The remaining 29 cases (72.5%) differed morphologically and hematologically from the other two groups in that they usually had a moderately high leukocyte count (average 27.9 x 10(3)/microliters), and abnormal cells showing a densely stained round nucleus and an inconspicuous nucleolus. Tartrate-resistant acid phosphatase reaction was weak, and their cells exhibited generally smooth or slightly irregular, cellular outlines in smears. The cells showed weak expression of surface immunoglobulin G (IgG) with kappa-chain predominance. CD25 antigen was not detected. Some of these findings resemble those of B-cell chronic lymphocytic leukemia, but the patients also had several important features of HCL. They had splenomegaly without significant lymphadenopathy. The abnormal cells were CD20+, CD11c+ and showed typical 'hairy morphology' under phase-contrast and scanning electron microscopy. Furthermore, spleen sections revealed diffuse infiltration by the abnormal cells in the red pulp. From these findings, we speculated that this group of patients constitute a distinct subtype of HCL which is commonly seen in Japan. We propose to term the disease as HCL Japanese variant.
 ...
PMID:Predominance of a distinct subtype of hairy cell leukemia in Japan. 842 71

A 33-year-old man was admitted to our hospital because of thrombocytopenia found on a periodic physical examination. Splenomegaly was recognized Peripheral blood showed WBC 4,510/microliters, Hb 12.5 g/dl, and Plt 40,000/microliters. Increased serum levels of acid phosphatase and angiotensin converting enzyme were observed on laboratory tests. Bone marrow aspirate revealed Gaucher cells. Decreased beta-glucosidase activity was demonstrated in blood leukocytes, cultured blood lymphocytes, and cultured bone marrow fubroblasts from the patient. His glucocerebrosidase genotype was T1448C/C1504T (L444P/R463C). Since neurological examination and skeletal X ray results were normal, Gaucher disease type 1 was diagnosed.
 ...
PMID:[Gaucher disease type 1, incidentally found on a periodic physical examination]. 868 74


<< Previous 1 2 3 4 5 Next >>