A Rare Case of Immune Mediated Hemolytic Anemia- Tuberculosis

Tuberculosis is one of the oldest diseases known to mankind. The disease still puzzles us with its varied clinical presentations and complications. Though tuberculosis is known to have many hematological manifestations, auto immune hemolytic anemia is extremely rare in tuberculosis. Here we report aninteresting case of tuberculosis presenting with auto immune hemolytic anemia. The treatment withanti tuberculous therapy is enough for the managing tuberculosis associated auto immune hemolyticanemia.

Evaluation of Demographic, Clinical and Laboratory Features

Background: Systemic lupus erythematosus (SLE) is a worldwide autoimmune disease. The disease has different etiologies, clinical and laboratory symptoms between different geographical and racial groups, and sufficient knowledge of the type of symptoms in each region can play a proper role in diagnosis and treatment. Objectives: This study was performed to evaluate demographic, clinical and laboratory features of patients with systemic lupus erythematosus in Kermanshah. Methods: This study is descriptive, analytical and cross-sectional. The files of 150 patients with lupus during 2016 - 2018 in Imam Reza hospital in Kermanshah were reviewed. Results: Data analysis showed that patients at the time of referral were with musculoskeletal symptoms 37.3% (56 individuals), cutaneous-mucosal 32% (48 individuals), constitutional 51.3% (77 individuals), renal 62% (93 individuals), cardiac 6.7% (10 individuals), neurological manifestations 17.3% (26 individuals), pulmonary involvement 37.3% (56 individuals), and Hematological 71.3% (107 individuals). The anti-nuclear antibody (ANA) in 60% (90 individuals), anti-double strand DNA Antibody (anti-ds DNA) in 35.4% (53 individuals), C-Reactive Protein (CRP) in 44.6% (67 individuals), lower level of normal C3 and C4 in 33.3% (50 individuals) and 11.3% (17 individuals), respectively, lupus anticoagulant in 13.3% (20 individuals), antibody citrullinated peptide anti-cyclic (anti-CCP) in 14.9% (22 individuals), anticardiolipin IgM and IgG, in 6% (9 individuals) and 9.3% (14 individuals) of patients respectively were observed. Also, anemia was observed in 34% (51 individuals), leukopenia in 22% (33 individuals), and thrombocytopenia in 30.7% (46 individuals). Abnormal ESR (erythrocyte sedimentation rate) was seen in 59.3% (89 individuals) of patients. Leukopenia in men and positive CRP in women were more common (P = 0.014, P = 0.004). Conclusions: Despite the diverse clinical and laboratory manifestations of SLE in different racial and geographical groups, paying attention to these differences in each region can effectively diagnose the disease. As in this study, hematological manifestations had a higher percentage in the population of lupus patients in Kermanshah.

Lupus-related vasculitis in a cohort of systemic lupus erythematosus patients

Objectives: This study aims to examine the frequency and clinical association of lupus-related vasculitis in patients with systemic lupus erythematosus (SLE). Patients and methods: We retrospectively analyzed medical records of a total of 565 SLE patients (42 males, 523 females; mean age: 32.7±9.5 years; range, 13 to 63 years) between January 2017 and February 2020. Demographic, clinical data, and laboratory data and treatment modalities applied were recorded. Lupus-related vasculitis and its different types were documented, and the patients with vasculitis were compared with those without vasculitis. Results: The mean disease duration was 8.9±6.3 years. Vasculitis associated with lupus was found in 191 (33.45%) patients. Cutaneous vasculitis was found in 59.2%, visceral vasculitis in 34.0%, and both in 6.8% of total vasculitis patients. The patients with vasculitis had a longer disease duration (p=0.01), were more likely to have juvenile onset (p=0.002), livedo reticularis (p<0.001), Raynaud's phenomenon (RP) (p<0.001), digital gangrene (p<0.001), thrombosis (p=0.003), and cranial neuropathy (p=0.004). The patients with vasculitis showed a higher prevalence of hypercholesterolemia (p=0.045), diabetes mellitus (p=0.026), higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) at disease onset (p<0.001), and Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (p=0.003) scores. They had more prevalent hematological manifestations (p<0.001), hypocomplementemia (p=0.007), received a higher cumulative dose of intravenous methylprednisolone (p<0.001), and had also more frequent cyclophosphamide (p=0.016) and azathioprine intake (p<0.001). In the logistic regression analysis, SLE vasculitis was independently associated with juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher scores of SLEDAI at disease onset (p<0.05). Conclusion: Juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher SLEDAI scores at disease onset may be associated with the development of vasculitis in SLE patients.

Pancytopenia and Kaposi sarcoma indicators of HIV infection diagnosis: A case report

During HIV infection, hematological manifestations are frequently associated with various pathogenic mechanisms, which require a multidisciplinary approach. We present the case of a 57-year-old patient with pancytopenia associated with Kaposi's sarcoma, as indicative manifestations of the acquired immunodeficiency syndrome. The late diagnosis of HIV infection, in the stage of severe immunosuppression, the association of opportunistic neoplasia, delayed oncological therapy and the development of immune reconstruction syndrome after starting antiretroviral therapy have contributed to the severe evolution and death of the patient.

Review on the Systemic Damage and Biochemical Alterations Associated with COVID-19 Infection

According to the WHO daily report, the world lost 4.3 million peoples and more than 203 million of the people were infected by the epidemic till August 10, 2021. Coronavirus Disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARSCoV-2) is a respiratory disease and it is a systemic infection with cardiovascular, pulmonary, gastrointestinal, neurological, and hematological manifestations, leading to death. Many biomarkers reflecting the main pathophysiological characteristics of the disease have been associated with the risk of developing severe disease. Biochemical changes like leukocytosis, neutrophilia, lymphopenia, cytokine storm, decreased albumin, increase in ALT, total bilirubin, LDH, and procalcitonin levels are significant predictors of ICU admitted COVID-19 patients. So, monitoring of biochemical parameters in COVID-19 patients is critical for assessing disease severity and progression as well as monitoring therapeutic intervention.

Hematological involvement in pediatric systemic lupus erythematosus: A multi-center study

Introduction: Systemic lupus erythematosus (SLE) may present with features of several systems, including hematological manifestations. In this study, we aimed to evaluate the characteristics of hematological involvement and assess possible associations and correlations in pediatric SLE patients. Method: This is a retrospective multi-center study. The medical records of pediatric SLE patients followed between January 2000 and June 2020 were analyzed. All children fulfilled the criteria of the Systemic Lupus International Collaborating Clinics. Results: The study included 215 children with SLE, 118 of whom had hematological manifestations. Concomitant renal involvement and low C3 levels were significantly more frequent in patients with hematological involvement ( p = 0.04, p = 0.008, respectively). Also, anti-cardiolipin, anti-beta-2-glycoprotein I (anti-β2 GP1), and anti-Sm antibody positivity, and the presence of lupus anticoagulant were more common in the group with hematological findings ( p = 0.001 for anti-cardiolipin antibody positivity and p < 0.001 for the positivity of anti-β2 GP1 antibody, anti-Sm antibody, and lupus anticoagulant). The most common hematologic abnormality was anemia (n = 88, 74.5%), with autoimmune hemolytic anemia constituting the majority (n = 40). Corticosteroids followed by IVIG were the mainstay of treatment. In patients resistant to corticosteroid and IVIG treatments, the most preferred drug was rituximab. Low levels of C3, high SLEDAI score, high incidence of renal involvement, and positive antiphospholipid antibodies were associated with hematological involvement in the univariate analysis. The presence of antiphospholipid antibodies and high SLEDAI score were independently associated with hematological involvement in multivariate analysis (OR: 4.021; 95% CI: 2.041–7.921; p < 0.001 and OR: 1.136; 95% CI: 1.065–1.212; p < 0.001). Conclusion: Hematological abnormalities are frequently encountered in pediatric SLE. Positive antiphospholipid antibodies and high SLEDAI scores were associated with hematological involvement.

Evan’s in a Case of Acute Myeloid Leukemia Post COVID-19 Infection

The Spectrum of Manifestations of COVID-19, A Pandemic Caused By Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), although emerged as a respiratory tract infection, is now regarded as a multi- system disease including hematological manifestations such as lymphopenia, thrombocytopenia, disseminated intravascular coagulopathy, COVID-19 associated coagulopathy and autoimmune cytopenia. It has been proposed that proinflammatory state induced by COVID-19 can lead to immune dysregulation and hence autoimmune cytopenia. Various case series have reported autoimmune hemolytic anemia (AIHA) post COVID-19 infection. We are reporting an unusual presentation of evan’s syndrome (Cold Autoimmune Hemolytic Anemia (CAIHA) with immune thrombocytopenia) secondary to COVID-19 in a case of Acute Myeloid Leukemia (AML) on consolidation chemotherapy with high dose cytarabine at our institute. As steroid did not seem to have any major response in our case, it is imperative to have a better understanding to guide the use of immunosuppression in autoimmune complications of SARS-CoV-2. We emphasize that one needs to be vigilant to look for these rare autoimmune manifestations, particularly in patients with underlying immune aberrancies due to their primary disease.

Avoiding misclassification of thrombotic primary antiphospholipid syndrome as systemic lupus erythematosus (SLE): What are the best-performing SLE classification criteria?

Background Systemic lupus erythematosus (SLE) and Primary Antiphospholipid Syndrome (PAPS) overlap clinical and immunological features. Therefore, misclassification of PAPS patients as SLE is a concern. The ACR/EULAR 2019 SLE classification has never been studied in PAPS. Objective To verify if the ACR/EULAR 2019 SLE classification can correctly classify a PAPS patient as not having SLE and compare its performance with the SLICC 2012 SLE classification. Methods: One-hundred thrombotic PAPS patients who fulfilled the Sidney criteria were consecutively screened and those who attended the inclusion criteria were submitted to ACR/EULAR 2019 and SLICC 2012 classifications. Results Sixty-seven PAPS patients were included in this study. The majority was female (89.6%) with median age at study inclusion of 45 years (35–53) and median PAPS disease duration of 13 years (8–19). PAPS correct classification was observed more often with ACR/EULAR 2019 than SLICC 2021 criteria (94.0% vs. 64.2%; p < 0.001). The 4 misclassified patients in ACR/EULAR 2019 were also misclassified in SLICC 2012. The comparison of misclassified patients to those correctly not classified as SLE resulted, for both criteria, in higher frequencies of hematological domain [ACR/EULAR 2019 (100% vs. 28.6%, p = 0.010) and SLICC 2012 (95.8% vs. 11.6%, p < 0.001)]. Further analysis of hematological manifestations revealed that for the ACR/EULAR 2019 leukopenia (100% vs. 22.2%, p = 0.004) and for the SLICC 2012 leukopenia/lymphopenia (91.7% vs. 7%, p < 0.001) were more frequent in misclassified group. Proteinuria (20.8% vs. 0%, p = 0.004) and low complement (45.8% vs. 20.9%, p = 0.033) were also more often observed in the incorrectly SLICC 2012 classified patients. Conclusion ACR/EULAR 2019 had high accuracy for distinguishing PAPS from SLE, whereas the SLICC 2012 incorrectly classified more than one third of the PAPS patients as having SLE.

Haematological abnormalities and pharmacotherapy in severe acute respiratory syndrome corona virus 2

The first case of SARS-CoV-2 (severe acute respiratory syndrome corona virus 2) was reported in Wuhan, China at the end of year 2019. It shows flu-like symptoms, but anosmia, fatigue, persistent cough and loss of appetite, that collectively might spot individuals with COVID-19. The aim of writing this review was to gather the information about blood abnormalities and pharmacotherapy for COVID-19 as a resource for healthcare professionals. A blood workup as well as continuous tracking hematological changes could divulge the risks of disease progression. The indirect indicators such as C-reactive protein (CRP), D-dimer, albumin, ferritin and LDH levels which are used as markers to estimate the severity of COVID-19 infection and prognosis. The most common hematological findings include lymphocytopenia, neutrophilia, eosinopenia, mild thrombocytopenia and less frequently, thrombocytosis. Clinical management includes prophylactic and therapeutic measures. Supportive care including supplemental oxygen and mechanical ventilatory support as and when indicated. Several class of drugs like anti-malarial, anti-viral, anti-inflammatory drugs are being used for the treatment and prevention of COVID-19. The target for development of most of the vaccine for COVID-19 is S protein of the corona virus. Various vaccines available for use across the globe are COVAX, Covishield, Moderna, Johnson and Johnson, Sputnik V, Novavax, Sinopharm, SinoVac. Serial monitoring of hematological manifestations is recommended and the treating doctor should stay vigilant and consider proper screening. The therapeutic intention is to decrease viral load and pharmacological thrombo-prophylaxis in high risk patients.