This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disorder that is frequently complicated by hematological manifestations such as hemolytic anemia, leukopenia and thrombocytopenia. 1,2 These disorders are included in the diagnostic criteria of SLE both in the earlier (1982), as well as in the revised criteria of the American College of Rheumatology (2003), and the 2012 Systemic Lupus International Collaborating Clinics (SLCC) criteria. 3-5 Thrombocytopenia (<100x10 9 /L) has been reported in 20% to 40% of patients with SLE 2,6-8 and is usually attributed to an autoimmune mechanism similar to that of idiopathic immune thrombocytopenia (ITP). It may be the first manifestation of lupus in up to 16% of patients, presenting months or as early as 10 years before diagnosis. 9,10 It can also be a complication of therapeutic agents such as azathioprine, methotrexate and rarely hydroxychloroquine. Severe thrombocytopenia (<20 to 50x10 9 /L according to study definition) is relatively rare, occurring in about 3-10% of patients. 11,12 Occasionally, thrombo-Cite this article as: Galanopoulos N, Christoforidou A, Bezirgiannidou Z. Lupus thrombocytopenia: pathogenesis and therapeutic implications.
BackgroundHyperhemolytic Syndrome or Hyperhemolytic Transfusion Reaction (HHTR), a life-threatening subset of Delayed Hemolytic Transfusion Reaction (DHTR) is characterized by destruction of both transfused and autologous erythrocytes evidenced by a fall in post transfusion hemoglobin below the pre-transfusion level.Case reportWe describe a case of DHTR due to anti-P1 alloimmunization manifesting with hyperhemolysis in a 30-year-old Greek Pomak woman with thalassemia intermedia (HbO-Arab/β-thalassemia), during the11th week of her first gestation. She was successfully managed with avoidance of further transfusions and administration of IVIG and corticosteroids.ConclusionA high index of suspicion for HHTR is of vital importance among clinicians especially since optimal methods for its prevention and treatment remain yet to be defined. Early recognition of HHTR leading to prompt cessation of additional transfusions and initiation of immunosuppressive treatment can be life-saving, especially in clinical settings where limited therapeutic options are available, such as in pregnancy.
The purpose of this review is to raise awareness about the frequently underappreciated association of blood donation with iron deficiency, and to describe methods for its prevention and management. Blood donors cannot expect any health benefits from the donation but have justified expectations of no harm. Iron deficiency without anemia (IDWA) and iron deficiency anemia (IDA) are common consequences of regular blood donation, and this activity is the most important factor affecting iron status in regular blood donors. Awareness of blood donation as a primary cause of sideropenia is surprisingly low among physicians. Blood donation screening identifies potential donors with IDA but is frequently inadequate to detect IDWA. For the assessment of body iron stores, plasma or serum ferritin, transferrin saturation (TSAT) and soluble transferrin receptors (sTfR) concentrations are the most widely used biochemical markers, although the percentage of hypochromic mature erythrocytes and the hemoglobin content of reticulocytes are also useful. IDWA can be prevented by limiting the total volume of blood collected, by iron deficiency screening and deferral of sideropenic donors, by prolonging the interdonation intervals, and by iron supplementation between donations. IDWA tends to be more prevalent in younger people, females, and high-intensity donors. A potentially effective strategy to address sideropenia in blood donors is serum ferritin testing, but this may lead to a higher rate of deferral. Most regular blood donors cannot replenish their iron deficit by an iron-rich diet alone and will benefit from low-dose oral iron administration with various commercially available products post-donation, a well-tolerated strategy. However, valid concerns exist regarding the possibility of worsening the iron overload in donors with undiagnosed hemochromatosis or masking the symptoms of a clinically important gastrointestinal hemorrhage or other underlying medical condition. Finally, educational efforts should be intensified to improve the awareness of blood donation as a primary cause of iron deficiency among physicians of all specialties.
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