scholarly journals Primary Autoimmune Hemolytic Anemia Due To Warm Reactive Autoantibodies: A Rare Case Report

2021 ◽  
Vol 8 (3) ◽  
pp. 119-123
Author(s):  
Dr. Abhijit Shinde ◽  
Dr. Sonal Shinde ◽  
Dr. Sushrut Kumar ◽  
Dr. Ramesh Kothari ◽  
Dr. Sneha Mhaske

Autoimmune hemolytic anemia (AIHA) is an acquired form of hemolytic anemia in which autoantibodies target red blood cell (RBC) membrane antigens, inducing cell rupture (lysis).  It affects both pediatric and adult populations, although its presentation in childhood is relatively rare, with the annual incidence estimated to be approximately 0.8 per 100,000 individuals under 18 years old [3]. Here we report  one such a rare case of autoimmune hemolytic anemia due to primary warm reactive autoantibodies in a 3 year old female child. As there was presence of hemolysis in peripheral blood smear &  other investigations also,  Direct coomb’s test was done & it came out to be positive which was suggestive of autoimmune hemolytic anemia, as following lab reports are suggestive of continuous destruction of RBC & after introduction of steroids parameters of  hemolysis came out to be normal suggestive of warm reactive autoantibodies type of AIHA. Clinically also patient improved & her urine colour also became normal after when prednisolone started. Patient also did not have any features of secondary causes of warm autoantibody like Systemic lupus erythematous, immunodeficiency disorders, ulcerative colitis & lymphoproliferative disorders so it was considered primary or idiopathic. W-AIHA tends to have a chronic course and is not expected to subside without treatment. It can be a fatal disease, with a mortality rate of up to 4% in children, either because of the acuity of the presentation or because of being refractory to treatment and requiring multiple lines of therapy with frequently associated toxicity [14]. Fortunately our patient responded to steroid therapy.

2021 ◽  
Vol 27 (2) ◽  
pp. 124-128
Author(s):  
Feda Anisah Makkiyah ◽  
Rahmah Hida Nurrizka

Objective and Importance. To illustrate the development of a rare case of spontaneous subdural hematoma (SDH)  secondary to aplastic anemia and conservative treatment of SDH. Clinical Presentation. A 43-year-old male complained of severe progressive headaches that starting from one month ago. His laboratory values showed pancytopenia and his peripheral blood smear showed no abnormalities except lack of the number of erythrocytes, leukocyte, and thrombocyte and we could not find any malignancy in the smear. He experienced headache,  disorder of balance and decrease of consciousness  CT imaging of the head showed  a 7.0 cm (2 cm thickness) left frontal-parietal subdural hematoma. Conclusion. Aplastic anemia is a rare case with manifested of subdural hematoma.


2020 ◽  
Vol 11 (SPL1) ◽  
pp. 1894-1897
Author(s):  
Varsha Gajbhiye ◽  
Shubhangi Patil (Ganvir) ◽  
Sarika Gaikwad

A 14-month female child came with complain of cholera like watery loose stool 10-12 times and vomiting 6-7 times, 24 hrs before admission. She was in severe dehydration, hypotension, unconscious with no recorded fever during her stay in hospital and no history of contact with COVID-19. Patient was COVID-19 positive Dehydration and hypotension was corrected, metabolic acidosis continued and eventually patient succumb due to multiple organ failure. This case report should arouse us to suspect COVID infection in every acute Gastroenteritis child who may not have any common symptoms as seen in COVID patient, also who have no history of significant contact with COVID positive patient in family. Some people with COVID-19 develop gastrointestinal symptoms either alone or with respiratory symptoms. Recently, researchers at Stanford University found that a third of patients they studied with a mild case of COVID-19 had symptoms affecting the digestive system. Another recent study Trusted Source published by researchers in Beijing found that anywhere from 3 to 79 percent of people with COVID-19 develop gastrointestinal symptoms.


2020 ◽  
Vol 27 (2) ◽  
pp. 83-86
Author(s):  
Z. Radonjić ◽  
B. Andrić ◽  
O. Šerbić ◽  
D. Mićić ◽  
M. Kuzmanović ◽  
...  

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5385-5385
Author(s):  
Waqas Ahmed ◽  
Kevin Monroe ◽  
James Essell ◽  
E. Randolph Broun

Abstract Introduction: Anemia is a common problem in patients with inflammatory bowel diseases (IBD), and its etiology is usually multifactorial. It can be produced by chronic blood loss, nutritional deficiencies, and drugs such as salazopyrine; however it can also due to auto immune hemolysis, which is a rare complication of IBD. We report a case of coombs positive autoimmune hemolytic anemia associated with ulcerative colitis both diagnosed at the same presentation. Case Report: A 32 year old man with no significant past medical history presented with complaint of dark colored urine, jaundiced skin and fatigue for 4 weeks. He also reported diarrhea mixed intermittently with blood for last few months. Physical exam was consistent with jaundice and anemia (pallor and icterus) with slightly palpable spleen. Initial lab work up showed Hb of 3.8 with normal platelet and WBC count, high reticulocytes count of 7% .LFT showed serum bilirubin of 3.6 (direct 0.4) with normal serum ALT and AST levels .Serum LDH was high (1032 U/l) while serum haptoglobin was low (0.11 mg/dl). Peripheral smear showed anisopoikilocytosis & spherocytosis. (See Figure 1) Further investigations revealed a positive direct Coombs test consistent with diagnosis of autoimmune hemolytic anemia. CT abdomen and pelvis showed mild splenomegaly & non-specific enlarged mesenteric lymph nodes. Colonoscopy revealed ulcerative pancolitis confirmed by histological findings of biopsies taken. Patient received PRBC transfusions and was started on steroids and mesalamine and was discharged on maintenance dose. His symptoms resolved in 4 weeks and Hb remained stable with no evidence of further hemolysis at 4 month follow up .Repeated CT abdomen & pelvis showed resolution of the lymphadenopathy. Figure 1: Peripheral Blood smear showing anisopoikilocytosis & spherocytosis. Figure 1:. Peripheral Blood smear showing anisopoikilocytosis & spherocytosis. Discussion: Autoimmune hemolytic anemia (AIHA) is a rare complication of IBD. The exact underlying pathogenesis of this association remains obscure; however it has been attributed to the production of cross reacting anti erythrocyte antibodies. In AIHA associated with IBD, corticosteroids are considered to be first line therapy and often cause remission of hemolysis along with treatment for IBD Immunomodulators and splenectomy has been used for patients with refractory AIHA. Colectomy done for fulminant colitis has also been reported to induce remission of AIHA. Further studies for long term follow up and pathogenesis of this association are warranted.


Author(s):  
Rahul Temani ◽  
Hemeshwar Harshvardhan ◽  
Ashwini Kumar Khicher ◽  
Priyanka Bansal

<p class="abstract">Blount’s disease is more common in Afro-Caribbean, obese, female child. Early walking have been associated with the disease process. Adult patients with Blount’s disease who received no treatment previously, are rarely reported across the other parts of the world and hence less discussed. The treatment of neglected cases of Blount’s disease often requires correction of varus deformity, internal tibial torsion and medial plateau depression. We are reporting one such patient seen at our hospital. A skeletally mature, obese (BMI 43.2), male patient with bilateral tibia vara was operated for hemi-plateau elevation combined with a valgus osteotomy. Fixation was achieved with plate and staples. Surgical correction of one such patient is technically demanding and more complicated. If left untreated , the main risk is premature osteoarthritis.</p>


2021 ◽  
pp. 416-419
Author(s):  
Khushboo Bhalla ◽  
Nagaraju Kamarthi ◽  
Sangeeta S Malik ◽  
Sumit Goel ◽  
Swati Gupta ◽  
...  

Tuberculosis (TB) is a chronic infectious granulomatous disease caused by the air-borne bacillus Mycobacterium tuberculosis and less frequently by other bacteria in the M. tuberculosis complex (Mycobacterium Bovis and Mycobacterium africanum). Tuberculous osteomyelitis of the condyle may present atypical clinical findings akin to temporomandibular joint arthritis or middle ear infections. A detailed clinical and radiographic examination aided by a histopathological and a microbiological diagnostic workup is the key to timely detection and administration of appropriate therapeutic regimens. A high degree of clinical suspicion is thus advocated in patients with such atypical presentations. We, hereby, are presenting a rare case of tuberculous osteomyelitis in a 15-year-old female child.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4817-4817 ◽  
Author(s):  
Jasmin Desai ◽  
Catherine Broome

Autoimmune hemolytic anemia (AIHA) is defined as the development of autoantibodies against red blood cell (RBC) antigens. AIHA is diagnosed in the presence of hemolysis and a positive direct antiglobulin test (DAT) for IgG and/or complement C3d. AIHA is classified as warm, cold or mixed based on the temperature at which the autoantibody is most active. The most clinically severe anemia is usually associated with AIHA in which C3d is detected on DAT. The classical complement pathway is activated in AIHA when antigen-autoantibody complexes on the RBC surface bind to the complement protein C1q. This ultimately results in the generation of C3b. C3b is both an opsonin and an additional proteolytic enzyme. C3b deposition targets the RBC for phagocytosis by macrophages of the reticuloendothelial system resulting in extravascular hemolysis. C3b can also continue activation of complement on the RBC membrane resulting in formation of the membrane attack complex (MAC) and cell lysis. C3b is degraded into C3d, which is identified by the DAT (Bartolmas 2015). Severe anemia and its immediate consequences, the difficulty identifying suitable units for acute RBC transfusion, the likelihood of causing additional hemolysis as well as concerns for stimulating additional antibody production with transfusion make these patients particularly challenging to treat. Current standard treatment consists of steroids with or without rituximab. This treatment paradigm does not, however, address the acute and urgent management of life threatening anemia in many of these patients. C1 esterase inhibitor (C1-INH) is a member of the serine protease inhibitor family and interacts with C1 esterase to block activation of the classical pathway of complement. Case reports have demonstrated that C1-INH can prevent C3-mediated lysis of PNH erythrocytes (DeZern 2014) and augment survival of transfused RBCs in a patient with DAT C3d positive autoimmune hemolytic anemia (Wouters 2013). We hypothesized that minimizing or inhibiting the generation of C3b with C1-INH in patients with either severe cold autoantibody AIHA or a mixed AIHA would rapidly reduce acute hemolysis. We report our clinical experience using a novel approach in the acute management of severe AIHA using the commercially available C1-INH, Berinert. The four patients we identified with either cold or mixed AIHA (Table 1) were between the ages of 58 and 63. All patients presented with clinically severe anemia and required urgent management. Three of the 4 had a co-morbid condition associated with AIHA. All patients received prednisone (1mg/kg) and the commercially available C1-INH at a dose of 20mcg/kg daily from the day of admission for a minimum of 6 days to a maximum of 20 days. Patients 2, 3, and 4 also received 4 weekly doses of rituximab during the course of treatment. The average increase in mean hemoglobin was 79% (presentation mean 4.5gm/dL- end of C1-INH mean 7.95gm/dL) during C1-INH administration. An average of 1.25(0-2) units of packed RBC per patient were transfused during C1-INH administration. Additional measures of ongoing hemolysis including mean LDH which decreased by an average of 72% (presentation mean 1358u/L to end of C1-INH mean 356u/L) and mean haptoglobin which increased in all patients from < 8mg/dL to 111mg/dL after C1-INH. Of note, patient 1 had cold agglutinin titers measured prior to C1-INH administration at 1:128 and repeated after two days of C1-INH administration revealed a decrease to 1:32. This retrospective review of patients demonstrates that utilizing C1-INH in conjunction with standard therapy is safe and results in rapid improvement of hemoglobin levels in patients with DAT/C3d positive AIHA. We hypothesize the inhibition of C1q-C4 interaction rapidly reduces C3b deposition on the RBC membrane decreasing both extra and intravascular RBC destruction. CI-INH therapy allowed successful management of these acutely ill patients with minimal RBC transfusions. We hypothesize that decreasing the exposure to additional antigenic stimulation may shorten the acute exacerbation of hemolysis. The optimal form and schedule of C1-INH therapy for AIHA remains to be determined. Based on these observations the role of inhibitors of the classical pathway of complement as both a single modality and in conjunction with standard therapy in the management of AIHA deserves further investigation. Table Table. Disclosures Broome: Alexion Pharmaceuricals: Honoraria; True North Therapeutics: Honoraria.


2014 ◽  
Vol 14 (2) ◽  
pp. 198-200
Author(s):  
Ahmedul Kabir ◽  
Aparna Das ◽  
Mohammad Shahidul Islam ◽  
Mohammad Shahin Masud ◽  
Fathima Aaysha Cader ◽  
...  

Visceral Leishmaniasis is a very common but neglected disorder in Bangladesh. It can remain subclinical or become symptomatic with an acute, sub acute or chronic course. Atypical presentations can be equally challenging to the clinician. Visceral leishmaniasis is associated with various autoimmune phenomena. Sometimes it can mimic any autoimmune disorders including Autoimmune Hemolytic Anemia (AIHA), SLE, and Rheumatoid Arthritis etc. Here, we describe a case of visceral leishmaniasis with Coombs’ positive autoimmune hemolytic anemia.DOI: http://dx.doi.org/10.3329/jom.v14i2.19686 J Medicine 2013, 14(2): 198-200


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