scholarly journals A case of pregnancy complicated with Evance syndrome and heparin-induced thrombocytopenia

2021 ◽  
pp. 91-99
Author(s):  
O.M. Naumchik ◽  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Underlying Disease ◽  
Evans Syndrome ◽  
Thrombotic Risk ◽  
Heparin Induced Thrombocytopenia ◽  
First Line Therapy ◽  
Successful Termination ◽  
Neonatal Thrombocytopenia

The simultaneous development of autoimmune hemolytic anemia and immune thrombocytopenia is known as Evans syndrome. This pathology is extremely rare during pregnancy, requires careful differential diagnosis and is associated with a high risk of hemorrhagic and thromboembolic complications. Treatment approaches for autoimmune hemolytic anemia and immune thrombocytopenia are similar, but in the case of autoimmune hemolytic anemia, thromboprophylaxis is mandatory. Heparin-induced thrombocytopenia is a serious adverse event of anticoagulation. One presents with a decrease in platelets but a paradoxical increase in thrombotic risk and mandates switching of the anticoagulant agent to a non-heparin one. Clinical case. We describe the case of pregnancy complicated with Evans syndrome and development of heparin-induced thrombocytopenia at 28 weeks. Treatment of the underlying disease was effective on the use of first-line therapy. Fondaparinux and vitamin K antagonist (warfarin) have been used to treat heparin-induced thrombocytopenia. Monitoring of the fetal condition is carried out with careful control of Doppler ultrasound of the middle cerebral artery. Successful change of anticoagulant, close surveillance and carefully selected therapy allowed to achieve a successful termination of pregnancy. A healthy female newborn with body weight 2450 g, 8–8 Apgar scores was vaginally delivered uneventful at the 38th week. There were neither neonatal thrombocytopenia nor anemia. During the year after birth, the woman's condition is not worse, the child develops according to age. An analysis of similar cases of pregnancy from the literature is described. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the author. Key words: thrombocytopenia, hemolytic anemia, pregnancy, Evans syndrome, heparin-induced thrombocytopenia.

scholarly journals Scrotal Pyoderma Gangrenosum Associated with Evans Syndrome

2018 ◽  
Vol 7 (9) ◽  
pp. 230
Author(s):  
Deng-Ho Yang ◽  
Meng-Yin Yang
Keyword(s):  
Emergency Room ◽  
Hemolytic Anemia ◽  
Pyoderma Gangrenosum ◽  
Autoimmune Hemolytic Anemia ◽  
Severe Pain ◽  
Immune Thrombocytopenia ◽  
Steroid Treatment ◽  
Evans Syndrome ◽  
History Of ◽  
Cutaneous Ulcer

Evans syndrome is a rare disorder with presentations of autoimmune hemolytic anemia and immune thrombocytopenia, in the absence of any underlying cause. Here, we reported a case with a history of Evans syndrome for seven years. A persistent scrotal ulcer with severe pain occurred for two weeks. He called at our emergency room because of a painful, necrolytic cutaneous ulcer over the scrotal region. A biopsy showed sterile dermal neutrophilia with lymphocytic vasculitis, and pyoderma gangrenosum was impressed. The patient received steroid treatment and recovery after one month.

scholarly journals NUDT15 polymorphism explains serious toxicity to azathioprine in Indian patients with chronic immune thrombocytopenia and autoimmune hemolytic anemia: a case series

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Anup J. Devasia ◽  
Raveen Stephen Stallon Illangeswaran ◽  
Infencia Xavier Raj ◽  
Biju George ◽  
Poonkuzhali Balasubramanian
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Case Series ◽  
Severe Toxicity ◽  
Toxic Side Effect ◽  
Case Presentation ◽  
Oral Ulcers ◽  
Asian Patients ◽  
Life Threatening

AbstractObjectivesAzathioprine (AZA) is a commonly used immunosuppressant in patients with autoimmune diseases. The toxic side effect to AZA (myelosuppression, hair loss, and oral ulcers) are highly unpredictable which can be life threatening if not identified earlier and dose adjustments made or the drug is withdrawn.Case presentationHere we report a case series of five patients with severe toxicity while on treatment with AZA for autoimmune hemolytic anemia (n=1) and Immune thrombocytopenia (n=4). The common thiopurine methyltransferase (TPMT) variants (TPMT*2, *3A, *3B) were not present in these patients. However, all these patients had the NUDT15 415C>T variant that has been reported to explain serious toxicity to thioguanine in Asian patients.ConclusionsOur report suggests pre-emptive genotype-based dosing of AZA could reduce adverse toxicity and hence better outcome.

A CASE OF EVANS SYNDROME WITH MIXED-TYPE AUTOIMMUNE HEMOLYTIC ANEMIA

2012 ◽  
Vol 58 (4) ◽  
pp. 539-546
Author(s):  
Takahiro Amamoto ◽  
Kouichi Egashira ◽  
Hiroyuki Kawano ◽  
Takanori Higashitani ◽  
Ken Ishimaru ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Mixed Type ◽  
Evans Syndrome

Rituximab Therapy for Treatment of Chronic Warm Autoimmune Hemolytic Anemia

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5386-5386
Author(s):  
Shylendra B Sreenivasappa ◽  
Margaret Clare Telfer
Keyword(s):  
Immunosuppressive Therapy ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Hemorrhagic Cystitis ◽  
Case Series ◽  
Complete Response ◽  
Cytotoxic Therapy ◽  
Gradual Improvement ◽  
First Line Therapy ◽  
Minimal Improvement

Abstract Autoimmune hemolytic anemia (AIHA) is a disorder in which auto antibodies are directed against red blood cells resulting in severe anemia. It is a relatively uncommon disorder with an estimated incidence of 1–3 cases per 100,000 persons per year. These disorders are classified as either warm AIHA or cold AIHA depending on the temperature at which the auto antibodies show maximal binding. First line therapy for patients (pts) with warm AIHA is corticosteroids, 20% achieve complete remission most patients require maintenance treatment. Splenectomy is the usual second line treatment. Cytotoxic and immunosuppressive medications can be tried. Rituximab a monoclonal antibody against CD 20 has also shown efficacy in pt with chronic AIHA. In this study we retrospectively studied 2 cases of chronic warm AIHA. Case 1 was a female diagnosed with AIHA at the age of 15 years with a history of vasculitis. AIHA had a relapsing and a remitting course. She was treated with steroids, splenectomy, cytotoxic therapy with cyclophosphamide and immunosuppressive therapy with azathioprine with no long-term remission. At 36 yr of age she had a relapse, steroids induced a minimal response, cyclophosphamide induced a partial response. She developed hemorrhagic cystitis hence cyclophosphamide was discontinued. Rituximab was administered at 375mg/m2 weekly for 3 doses with minimal response. After 4 weeks she was given 3 more doses of weekly rituximab with minimal response. 2 weeks after the last Rituximab dose, pt developed pancytopenia, gram-negative sepsis and died. Case 2 was also a female who was diagnosed with AIHA at the age of 21 years, etiology was considered idiopathic. She had a relapsing remitting course as well. She had been treated with steroids, splenectomy, cytotoxic therapy and immunosuppressive therapy. At 41 years of age she relapsed, this episode was, not responsive to steroids, Rituximab 375mg/m2 was infused weekly for 3 weeks with minimal improvement, after 6 weeks, 3 more doses of Rituximab were infused. She had a gradual improvement of her hemoglobin over 4 months and pt has been in remission since. She has had a sustained response for over 24 months. There have been a few case series published about the effects of Rituximab in pt with warm AIHA, with an estimated complete response ranging from 40 – 100%. The toxicities of this therapy in this pt population has been not been well documented. We conclude that Rituximab must be used cautiously in pt with AIHA. Prospective Randomized clinical trails are needed to assess the efficacy of Rituximab in pt with warm AIHA.

scholarly journals The Challenge of Transfusion of Patients Infected with HIV/AIDS

2019 ◽  
Vol 4 (2) ◽  
Keyword(s):  
Human Immunodeficiency Virus ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Hypercoagulable State ◽  
Thrombotic Risk ◽  
Heparin Administration ◽  
Immunodeficiency Virus ◽  
Positive Crossmatch ◽  
Hiv Negative ◽  
Hiv Aids

The transfusional support of human immunodeficiency virus-infected patients is a challenge both for the clinical physician and for the blood services, either because of the immunohematological problems or the microbiological/thrombotic risk associated. The immunohematological risk caused by positive crossmatch is resolved by autologous adsorption; if the patient was recently transfused, the adsorption will be homologous. The thrombotic risk (due to hypercoagulable state) is resolved by pretransfusion heparin administration and leukoreduction only in autoimmune hemolytic anemia cases; and the presumed microbiological risk is similar to HIV-negative patients.

scholarly journals Autoimmune Cytopenias and Covid-19 Vaccination: Relapse and Suggested Treatment

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4141-4141
Author(s):  
Gregorio Campos-Cabrera ◽  
Francisco-Gerardo Torres-Salgado ◽  
Salvador Campos-Cabrera ◽  
Jose-Luis Campos-Villagomez ◽  
Virginia Campos-Cabrera
Keyword(s):  
Complete Remission ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
De Novo ◽  
Conflicts Of Interest ◽  
High Dose ◽  
Evans Syndrome ◽  
Autoimmune Thrombocytopenia ◽  
Platelet Counts ◽  
Autoimmune Cytopenias

Abstract Introduction: There are "de novo" and relapsed autoimmune diseases in patients with COVID-19 that includes autoimmune thrombocytopenia, Evans syndrome and autoimmune hemolytic anemia among others (Hematology 2021;26:225-239 and Curr Rheumatol Rev 2021;17:193-204). There is scanty material about relapse of autoimmune hematological diseases after vaccination for COVID-19 (Blood Adv 2021;13:2794-2798). Material and methods: Adult patients 18 years or older with autoimmune thrombocytopenia, Evans syndrome and autoimmune hemolytic anemia who completed SARS-Cov2 vaccination. Results: Between December 2020 and June 2021 there were identified 53 patients with autoimmune hematological disease that completed SARS-Cov2 vaccination. Thirty-six with autoimmune thrombocytopenia, all were preexisting. Twelve with autoimmune hemolytic anemia, 5 secondaries to previous COVID-19 and 7 preexisting. Five with Evans syndrome, all preexisting. Twenty-three patients with autoimmune thrombocytopenia did not develop any fall in the platelet count. Ten patients had a fall of 50 % from basal counts and recovered spontaneously. Three patients developed counts below 30,000 with purpuric symptoms and needed treatment that consisted in two courses of dexamethasone 40 mg daily for four days every three weeks; all patients reached complete remission without any further treatment. All patients with Evans syndrome developed hemolysis and low platelet counts. Two patients maintained Hb levels above 10 and platelet counts above 50,000; both patients had spontaneously recovery. Three patients developed Hb levels below 7 with anemic syndrome and platelets below 50,000 but without purpuric syndrome; they received the same treatment as patients with autoimmune thrombocytopenia and reached complete remission too. All five patients with autoimmune hemolytic anemia secondary to COVID-19 developed Hb levels below 7 with anemic symptoms and needed treatment as described. The remaining 7 patients with preexisting autoimmune anemia developed hemolysis; five with Hb levels above 7 and recovery without any treatment; two had Hb levels below 7 and received the same treatment with full recovery and complete remission. Conclusions: Autoimmune cytopenias can be trigger by vaccines and viral infections by involving molecular mimicry and circulating immune complexes, including SARS-Cov2. The viral protein spike from SARS-Cov2 has mimicry between the Ankyrin-1 in the erythrocyte surface, and has been linked as one of the pathogenesis pathways of autoimmune hemolytic anemia secondary to COVID-19 (Br J Haematol 2020;190:e92-e93 and Blood 2020;136:suppl8,138001). Relapse of autoimmune cytopenias after vaccination with SARS-Cov2 involves stimulation of autoantibodies production from preexisting B cells. Although relapses were observed in the three kinds of patients, all with hemolytic component developed a drop in the hemoglobin levels, most of them needed treatment. It is important to notice that patients with hemolytic autoimmune anemia secondary COVID-19 had severe relapse, event that support the mimicry mentioned lines above. It is important to follow up closely this kind of patients after SARS-CoV2 vaccination, we suggest weekly complete blood counts, and a short courses of high dose dexamethasone can induce curable responses if treatment is advised. Disclosures No relevant conflicts of interest to declare.

scholarly journals Difficulties of anemia diagnosis in patients with B-cell chronic lymphocytic leukemia

2021 ◽  
Vol 16 (2) ◽  
pp. 40-47
Author(s):  
I. S. Piskunova ◽  
T. N. Moiseeva ◽  
L. S. Al-Radi ◽  
L. V. Plastinina ◽  
S. R. Goryacheva
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
B Cell ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Lymphocytic Leukemia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Cell Chronic Lymphocytic Leukemia

Cytopenia commonly occurs in case of chronic lymphocytic leukemia. It can either precede the diagnosis of chronic lymphocytic leukemia or appear at any time during the disease. Autoimmune hemolytic anemia, immune thrombocytopenia, and partial red cell aplasia are most often found among cytopenias in chronic lymphocytic leukemia. At the same time, the development of cytopenia may be associated with the displacement of normal hematopoiesis cells by tumor lymphocytes. It is very important to accurately diagnose and identify the cause of cytopenia in chronic lymphocytic leukemia, since the prognosis and therapy differ significantly.

scholarly journals The role of rituximab in adults with warm antibody autoimmune hemolytic anemia

Blood ◽  
2015 ◽  
Vol 125 (21) ◽  
pp. 3223-3229 ◽  
Author(s):  
Daan Dierickx ◽  
Alain Kentos ◽  
André Delannoy
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Randomized Study ◽  
Line Treatment ◽  
Second Line ◽  
First Line ◽  
Second Line Therapy ◽  
First Line Therapy ◽  
Line Therapy

Abstract Warm antibody hemolytic anemia is the most common form of autoimmune hemolytic anemia. When therapy is needed, corticosteroids remain the cornerstone of initial treatment but are able to cure only a minority of patients (<20%). Splenectomy is usually proposed when a second-line therapy is needed. This classical approach is now challenged by the use of rituximab both as second-line and as first-line therapy. Second-line treatment with rituximab leads to response rates similar to splenectomy (∼70%), but rituximab-induced responses seem less sustained. However, additional courses of rituximab are most often followed by responses, at the price of reasonable toxicity. In some major European centers, rituximab is now the preferred second-line therapy of warm antibody hemolytic anemia in adults, although no prospective study convincingly supports this attitude. A recent randomized study strongly suggests that in first-line treatment, rituximab combined with steroids is superior to monotherapy with steroids. If this finding is confirmed, rituximab will emerge as a major component of the management of warm antibody hemolytic anemia not only after relapse but as soon as treatment is needed.

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