Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The present multicenter study was undertaken to confirm whether filgrastim/recombinant human granulocyte colony stimulating factor (rhG-CSF) could mobilize residual multipotential stem cells by its G0-shortening effect in patients with aplastic anemia (AA) and induce a multilineage response. Twenty-seven patients with acquired severe or moderate AA received long-term administration (2 to 12+ months) of rhG-CSF in doses from 100 to 400 micrograms/body/day by s.c. injection or 250 to 1,500 micrograms/body/day by i.v. infusion. Twenty-six out of the 27 evaluable patients showed a substantial increase in neutrophils associated with a recovery of myeloid precursors in bone marrow within one month of therapy. Interestingly, 10 out of the 27 patients showed a dramatic improvement in severe anemia after two to ten months of therapy. Moreover, severe thrombocytopenia improved after two to four months of therapy in three out of these ten patients accompanied by a significant increase in megakaryocytes in bone marrow. Clonal cultures of bone marrow cells revealed a recovery in myeloid as well as erythroid precursors in most of these ten patients. In two patients who showed a trilineage response, mixed and megakaryocyte colony formations also recovered. These results suggest that long-term administration of rhG-CSF mobilizes myeloid, erythroid, megakaryocyte and multipotential progenitor cells and induces a multilineage response in some patients with AA.
 ...
PMID:Multilineage response in aplastic anemia patients following long-term administration of filgrastim (recombinant human granulocyte colony stimulating factor). 750 23

A sprightly 79-year-old woman was treated for high blood pressure with indapamide (2.5 mg/day) and the angiotensin converting enzyme (ACE) inhibitor lisinopril (5 mg/day). About 12 months after starting treatment a blood count carried out because of a syncopal attack revealed pancytopenia (haemoglobin 3.3 g/dl, erythrocytes 1.0 x 10(6)/microliters, leucocytes 1100/microliters, platelets 8000/microliters). Until then the blood count had been unremarkable. The bone marrow showed severe hypoplasia of all three cell lines with reactive plasmocytosis. Malignant cells were not present. The patient received a total of nine units of erythrocytes and seven units of platelets. Her care included reverse barrier nursing and antibiotic treatment. She was also given high dose steroid therapy (methylprednisone up to 150 mg/day) and granulocyte colony stimulating factor (filgrastim 300 micrograms/day subcutaneously for 25 days), and after a latent period of several weeks juvenile myeloid precursors reappeared in the blood. Before discharge from hospital the results rose to subnormal levels without further transfusions (haemoglobin 8.5/dl, erythrocytes 3.1 x 10(6)/microliters, leucocytes 3900/microliters, platelets 21.000/microliters). In the bone marrow, all three cell lines were beginning to recover. The final diagnosis was incompletely reversible pancytopenia resulting from secondary aplastic anaemia during ACE inhibitor therapy.
 ...
PMID:[Severe pancytopenia in old age after 12-month ACE inhibitor therapy]. 751 41

The basic characteristics of granulo-macrophage colony stimulating factor (GM-CSF) and granulocyte colony stimulating factor (G-CSF) are reviewed. The structure of the proteins and genes have been clarified. The clinical use of these hematopoietic growth factors are also discussed. Diseases for which these growth factors will be effective are follows, the granulocytepenic state after anti-cancer chemotherapy, aplastic anemia, bone marrow transplantation and so on. As these growth factors increase the granulocyte function, these proteins will be effective on the immunodifective state.
 ...
PMID:[GM-CSF.G-CSF]. 753 64

Recombinant human granulocyte colony stimulating factor (rHuG-CSF) has been used for several years in clinical haematology and it is now routinely employed to prevent or treat chemotherapy-induced neutropenia. rHuG-CSF is also administered after autologous or allogeneic bone marrow transplantation (BMT), since it can significantly shorten the duration of neutropenia. However, probably its main use at the moment is to facilitate the collection of peripheral blood stem cells (PBSC) from patients with lymphoma, myeloma and breast cancer. Within controlled trials, it is also used as an adjunct to immunosuppression for patients with aplastic anaemia. rHuG-CSF has a number of other potential uses such as increasing the numbers of progenitor cells for transplantation by in vivo and/or ex vivo amplification; treatment of non-neutropenic infections post transplant, and prophylaxis and treatment of cytomegalovirus infections. In the future, autologous stem cell transplants may be performed in the outpatient department thus expanding the use of PBSC transplantation to disease areas not previously considered suitable for such myelosuppressive treatment.
 ...
PMID:The clinical benefits of recombinant human granulocyte colony stimulating factor in the treatment of cancer patients. 753 69

A 19-year-old male who suffered from severe aplastic anemia had been treated with granulocyte colony stimulating factor (G-CSF) from September 1991. Marked increase of hematopoietic cells in his bone marrow was observed, and maintenance administration of G-CSF was continued. 15 months later, myeloblasts with nuclear abnormality increased, and 22 months later, myeloblasts with chromosomal abnormality presenting 46, XY, -7, +21 exceeded 20%, and aplastic anemia seemed to be transformed into refractory anemia with excess of blasts in transformation (RAEB in T). The usefulness of G-CSF in the treatment of aplastic anemia is now established, but there are some reports questioning the effect of long-term administration, especially transformation to MDS with monosomy 7. Leukemic transformation from aplastic anemia is very complex, but in some cases, long term administration of G-CSF may affect the natural course and may lead to the earlier development of leukemia.
 ...
PMID:[RAEB in T with monosomy 7 after treatment of severe aplastic anemia with long term G-CSF]. 754 Feb 27

The effect of KW-2228, a derivative of recombinant human granulocyte colony stimulating factor, on neutropenia in children was studied in 23 cases of aplastic anemia, 13 cases of chronic benign neutropenia, 6 cases of congenital neutropenia (Kostmann type), 2 cases of cyclic neutropenia, 2 cases associated with glycogenosis type Ib and 1 cases associated with immune deficiency. KW-2228 was administered at 1-8 micrograms/kg subcutaneously and at 2-16 micrograms/kg intravenously. As a principle, the administration was started at low doses and continued for 7-28 days increasing the doses in the cases who didn't respond to the treatment well. The response rate of all the cases by the physicians in charge was 81. 8% (36/44). The mean absolute neutrophil count was increased from 304 to 1,300/microliters in aplastic anemia, from 204 to 3,027/microliters in chronic benign type, from 125 to 2,193/microliters in Kostmann type, and from 360 to 2,007 microliters in others. KW-2228 did not induce any noteworthy serious side effects. These results indicated that KW-2228 is a useful drug to treat neutropenia in children.
 ...
PMID:[Clinical study of recombinant human granulocyte-colony stimulating factor (KW-2228) in pediatric field. 1. Effectiveness on neutropenia by various causes and safety]. 768 91

Although aplastic anemia (AA) is well known to precede acute leukemia, commonly acute myeloblastic leukemia, an adult case of acute lymphoblastic leukemia (ALL) preceded by aplasia similar to severe AA is very rare. An 18-year-old female was admitted to our hospital for scrutiny of pancytopenia in October 3, 1991. She was diagnosed as severe AA because of severe bone marrow hypoplasia and no abnormal or dysplastic cell. Recovery of hematopoiesis was achieved by pulse therapy of methylprednisolone combined with granulocyte colony stimulating factor (G-CSF). 3 months after AA onset, she was readmitted because of high fever and paralytic ileus. Then, she was diagnosed as ALL (L1; common ALL) and was successfully treated by remission induction chemotherapy. Retrospectively, this case was atypical AA with respect to serum iron and LDH level.
 ...
PMID:[An adult case of acute lymphoblastic leukemia preceded by aplasia resembling aplastic anemia]. 768 95

Aplastic anaemia (AA) of the chronic type with severe cytopenia is very frequently a difficult therapeutic problem. Patients with granulocyte values below 0.5 G/l are threatened by infections, incl. sepsis possibly with a fatal outcome. If the pool of stem cells for granulocytes is not completely exhausted and can respond to growth factors, these patients can be treated either chronically and/or in risk situations (e.g. injury, surgery) with preparations of the type of a recombinant, granulocyte colony stimulating factor (rhG-CSF), or granulocyte and monocyte colony stimulating factor (rhGM-CSF). The authors present a review of diagnostic and therapeutic algorithms in patients with the AA syndrome and summarize their own experience with the preparation Neupogen Roche (rhG-CSF).
 ...
PMID:[G-CSF (Neupogen Roche) in the treatment of patients with chronic aplastic anemia with severe neutropenia]. 857 1

A male patient with severe aplastic anemia was admitted for bone marrow transplantation. While waiting for a donor, high doses of methylprednisolone, anabolic steroid and granulocyte colony stimulating factor were given without response. Deferoxamine was administered for prophylaxis of hemochromatosis because of high level of ferritin. Acute right lower abdominal pain and pyrexia developed. A diagnosis of acute appendicitis was made and appendectomy was performed. The histopathological examination of the resected appendix revealed necrotizing hemorrhagic appendicitis with numerous hyphae of Mucorales. Though anti-fungal agent (amphotericin B) administration was continued, he subsequently developed ileo-cecal abscess and eventually died due to myoglobinuric nephropathy caused by extensive necrosis of the iliopsoas muscle. Autopsy revealed dissemination of hyphae of Mucorales in lungs, kidneys, large vessels and muscle of the bilateral lower limbs. Systemic vascular invasion and embolization of fungal hyphae were also observed. However, culture of exudate sampled from ileocecum yielded no Mucorales. It was emphasized that antemortem diagnosis and effective anti-fungal treatments are essential for the management of intestinal mucormycosis. The relation ship between mucormyocosis and deferoxamine was also discussed.
 ...
PMID:[Acute appendicitis caused by mucorales in a patient with severe aplastic anemia: report of an autopsy case]. 885 34

About 30% of patients with severe aplastic anaemia (SAA) unresponsive to one course of immunosuppressive (IS) therapy with antithymocyte or antilymphocyte globulin can achieve complete or partial remission after a second IS treatment. Among various second-line treatments, rabbit ATG (r-ATG) could represent a safe and effective alternative to horse ALG (h-ALG). In a multicentre study, 30 patients with SAA (17 males and 13 females, median age 21 years, range 2-67) not responding to a first course with h-ALG plus cyclosporin (CyA) and granulocyte colony stimulating factor (G-CSF), were given a second course using r-ATG (3.5 mg/kg/d for 5 d), CyA (5 mg/kg orally from day 1 to 180) and G-CSF (5 microg/kg subcutaneously from day 1 to 90). The median interval between first and second treatment was 151 d (range 58-361 d). No relevant side-effects were observed, but one patient died early during treatment because of sepsis. Overall response, defined as transfusion independence, was achieved in 23/30 (77%) patients after a median time of 95 d (range 14-377). Nine patients (30%) achieved complete remission (neutrophils >/=2.0 x 109/l, haemoglobin >/=11 g/dl and platelets >/=100 x 109/l). The overall survival rate was 93% with a median follow-up of 914 d (range 121-2278). So far, no patient has relapsed. Female gender was significantly associated with a poorer likelihood to respond (P = 0.0006). These data suggest that r-ATG is a safe and effective alternative to h-ALG for SAA patients unresponsive to first-line IS treatment.
 ...
PMID:Rabbit antithymocyte globulin (r-ATG) plus cyclosporine and granulocyte colony stimulating factor is an effective treatment for aplastic anaemia patients unresponsive to a first course of intensive immunosuppressive therapy. Gruppo Italiano Trapianto di Midollo Osseo (GITMO) 1058 20


1 2 3 Next >>