Home Contact Sitemap

eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease neutropenia
Comorbidity C0002878|hemolytic anemia
Sentences 5
PubMedID- 21772952 Evans syndrome (es) is an autoimmune disorder characterized by thrombocytopenia and/or neutropenia associated with hemolytic anemia (aha).1–3 it is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymph-proliferative disorders.4 standard treatment consists of transfusions, corticosteroids and immunoglobulins (ivig); second-line treatment in es evolved over the last decade, and includes at present anti-cd20 rituximab, vincristine, alkalizing agents, mycophenolate mofetil (mmf) or cyclosporine (cya).5–6,8–14 long-term remissions induced by splenectomy are less frequent than in uncomplicated immune thrombocytopenia purpura (itp).
PubMedID- 24198852 This includes hemolytic anemia with reticulocytosis, leucopenia (<4.0x10⁹/l) or lymphopenia (<1.5x10⁹/l) on two or more occasions, or thrombocytopenia (<100x10⁹/l) in the absence of offending drugs [4,5].
PubMedID- 26063969 hemolytic anemia with reticulocytosis or leukopenia <4.000/mm3 on ≥2 occasions or lymphopenia <1.500/mm3 on ≥2 occasions or thrombocytopenia <100.000/mm3 in the absence of offending drugs.
PubMedID- 21306603 Records of clinical and laboratory findings during the study period were collected as follows: hemolytic anemia with reticulocytosis, leucocytopenia (< 4000 cells/mm3) and lymphopenia [absolute lymphocytic count (alc) <1,500 cells/mm3 ] present in 2 or more occasions, thrombocytopenia (< 100,000 cells/mm3) in the absence of offending drugs, abnormal urinalysis (proteinuria 0.5 g/24 h or >3+ if quantitative evaluation was not done, and/or casts, hematuria >5 rbcs/hpf or pyuria >5 wbcs/hpf in the absence of infection), increased creatinine (> 1 mg/dl), low c3, increased erythrocyte sedimentation rate (esr) (> 20 mm/hr).
PubMedID- 24864270 This ongoing registry had recruited 2170 chinese sle patients who fulfilled the sle classification criteria revised by the american college of rheumatology (acr) in 1997 [9] during the period between april 2009 and february 2010. patients were required to fulfill at least 4 of the following 11 criteria: (1) malar rash; (2) discoid rash; (3) photosensitivity; (4) oral or nasopharyngeal ulceration; (5) nonerosive arthritis involving 2 or more peripheral joints; (6) pleuritis or pericarditis; (7) nephropathy: persistent proteinuria > 0.5 grams per day or cellular casts; (8) neurologic involvement: seizures or psychosis in the absence of offending drugs or known metabolic derangements; (9) hematologic involvement: hemolytic anemia with reticulocytosis or leukopenia (<4,000/mm3 on ≥2 occasions) or lymphopenia (<1,500/mm3 on ≥2 occasions) or thrombocytopenia (<100,000/mm3) in the absence of offending drugs; (10) immunologic disorder: antibody to native double-stranded dna in abnormal titer or presence of antibody to sm nuclear antigen or positive finding of antiphospholipid antibodies; (11) positive antinuclear antibody.

Page: 1