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עמוד בית
Sat, 18.05.24

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March 2023
Sergei Elber-Dorozko MD, Yackov Berkun MD, Abraham Zlotogorski MD, Alexander Maly MD, Ariel Tenenbaum MD

IgA vasculitis, formerly known as Henoch–Schönlein purpura (HSP), is the most common systemic vasculitis in children. It is defined as palpable purpura in the absence of coagulopathy or thrombocytopenia and one or more of the following criteria: abdominal pain, arthritis or arthralgia, biopsy of affected tissue demonstrating predominant IgA deposition, and renal involvement with proteinuria and hematuria or red cell casts [1].

November 2022
Izabella Elgardt MD, Or Carmi MD, Yair Levy MD

At the end of 2019, the world faced a new virus–coronavirus disease 2019 (COVID-19)–which quickly became a pandemic. It has become clear that the COVID-19 virus can affect various body systems. Over time, we are finding more and more diverse manifestations of the course of the disease itself, its consequences, and complications. There have been several studies and reviews describing circulating antibodies in patients infected with COVID-19 (e.g., antinuclear antibodies [ANA], anti-cardiolipin, anti-B2 glycoprotein, perinuclear anti-neutrophil cytoplasmic antibodies [p-ANCA], cytoplasmic ANCA [c-ANCA]). The development of autoimmune disorders has been reported (e.g., Graves' disease, systemic lupus erythematosus (SLE), immune thrombocytopenia [ITP], diabetes mellitus [DM] type 1, psoriasis). There are descriptions of COVID-19 associated vasculitis include Kawasaki-like symptoms in children and immunoglobulin A (IgA) vasculitis in children and adults [1].

May 2022
Arthur E. Frankel MD, Dennis Wylie PhD, Bjoern Peters PhD, Daniel Marrama BS, and Chul Ahn PhD

Background: Secondary immune thrombocytopenic purpura (ITP) associated with coronavirus disease 2019 (COVID-19) is a rare but serious complication of the pandemic. Diagnostic criteria include clinical and laboratory findings. Early treatment is often effective, but rare severe bleeding and death can occur. An autoimmune mechanism is likely.

Objectives: To determine a role for molecular mimicry in producing disease.

Methods: Hexapeptide and heptapeptide matches between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and platelet N-glycosylated proteins and other human proteins were assessed.

Results: Shared viral and platelet glycoprotein peptides were found. Copy frequency of these peptides in the human proteome was low for many of the candidate molecular mimics.

Conclusions: The data support a contribution of molecular mimicry in COVID-19 ITP autoimmunity and offer avenues for in vitro diagnostic assay development. The continuation of the pandemic necessitates additional understanding of COVID-19 ITP as well as studies on diagnosis and mitigation.

 

June 2021
Paula David MD, Arad Dotan, Naim Mahroum MD, and Yehuda Shoenfeld MD FRCP MaACR
November 2017
Szilvia Szamosi MD, Nóra Bodnár MD, Boglárka Brugós MD, Tibor Hortobágyi MD, Gábor Méhes MD, Zoltán Szabó MD, Edit Végh MD, Ágnes Horváth MD, Zoltán Szekanecz MD, Attila Szűcs MD and Gabriella Szűcs MD
December 2013
July 2011
G.Y. Stein, D. Blickstein, J. Orlin, G. Sarig and A. Inbal
 

Acquired thrombotic thrombocytopenic purpura (TTP) is an uncommon disease in adults, characterized by fever, neurological manifestations, microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, and the presence of antibodies against the enzyme ADAMTS13. Treatment with plasmapheresis has increased the survival from 10% to more than 90%. Still, there is a subset of patients with resistant TTP who fail to respond to plasmapheresis or remain dependent on this procedure. There is mounting evidence that rituximab may play an important role in remission induction of resistant/relapsing TTP; however, the extent of the remission is unknown. We present here four patients with chronic-relapsing TTP who responded favorably to rituximab. All four patients achieved prolonged remission of 23 to 82 months after the treatment.  One patient relapsed 6 years after the initial treatment with rituximab and re-entered remission following retreatment.

 

April 2011
O. Eshach Adiv, Y. Butbul, I. Nutenko and R. Brik

Intussuception is the most common cause of intestinal obstruction in early childhood. The cause of most intussusceptions is unknown but it can complicate the course of Henoch-Schonlein purpura (HSP) as a result of the vasculitic process. Familial Mediterranean fever (FMF), a most common disease in Israel is also associated with HSP. In a few patients, particularly in children, HSP has been reported to precede the diagnosis of FMF. We describe two patients with an unusual clinical course of severe abdominal pain as a result of intusucception. The correlation between intusucception, HSP and FMF are discussed.
 

May 2008
L. Barski, E. Rabaev, I. Sztarkier, J. Delgado, A. Porath, and A. B. Jotkowitz
October 2007
October 2006
M. Shtalrid, L. Shvidel, E. Vorst, E.E. Weinmann, A. Berrebi and E. Sigler
 Background: Post-transfusion purpura is a rare syndrome characterized by severe thrombocytopenia and bleeding caused by alloimunization to human platelet specific antigens following a blood component transfusion. The suggested incidence is 1:50,000–100,000 transfusions, most often occurring in multiparous women. The diagnosis is not easy because these patients, who are often critically ill or post-surgery, have alternative explanations for thrombocytopenia such as infection, drugs, etc.

Objectives: To describe patients with initially misdiagnosed PTP[1] and to emphasize the diagnostic pitfalls of this disorder.

Patients and Results: During a period of 11 years we have diagnosed six patients with PTP, four women and two men. The incidence of PTP was approximately 1:24,000 blood components transfused. We present the detailed clinical course of three of the six patients in whom the diagnosis was particularly challenging. The patients were initially misdiagnosed as having heparin-induced thrombocytopenia, systemic lupus erythematosus complicated by autoimmune thrombocytopenia, and disseminated intravascular coagulation. A history of recent blood transfusion raised the suspicion of PTP and the diagnosis was confirmed by appropriate laboratory workup.

Conclusions: PTP seems to be more frequent than previously described. The diagnosis should be considered in the evaluation of life threatening thrombocytopenia in both men and women with a recent history of blood transfusion.


 





[1] PTP = post-transfusion purpura


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