Impending splenic rupture as indicator of atypical chronic lymphocytic leukemia: A case report from Saudi Arabia.

BACKGROUND: Chronic lymphocytic leukemia (CLL) is one of the hematological malignancies in which the bone marrow overproduces mature, dysfunctional lymphocytes. Affected lymphocytic cells can affect the lymph nodes, spleen, liver, bone marrow, and rarely other organs. Spontaneous rupture of the spleen is a rare health condition, with a few cases caused by CLL. Since the preliminary presentation of either impending or real splenic rupture is unusual and requires recording and reporting, this case report was developed. CASE REPORT: A 55-year-old male patient presented with a significant weight loss of 20 kg, loss of appetite, fatigue, abdominal pain, and early satiety. Clinical examination revealed massive splenomegaly 22 cm below the costal margin, otherwise unremarkable on systemic examination. The size and dimensions of the spleen were confirmed by computed tomography of the body. The patient underwent a bone marrow biopsy, which was suggestive of atypical CLL. Due to the rapid progression of the size of the spleen and the pattern of impending rupture of the spleen, an emergency splenectomy was performed, and the histopathological report confirmed low-grade B-cell non-Hodgkin's lymphoma, mostly typical of CLL. CONCLUSION: Given the rarity of cases, continuous data recording and reporting is required to enrich the scientific literature and share experiences from case to case. This will create a complete picture of this health condition across diverse regions and countries.

Coronavirus disease 2019 (COVID-19) in special groups: A single-center experience in sickle cell disease patients in Saudi Arabia.

BACKGROUND: Sickle cell disease (SCD) is a group of hereditary diseases, inherited as autosomal recessive disorder, which causes mutation in the ß-globin gene. As a result, there is a change in the sixth amino acid from glutamic acid to valine. The affected red blood cell is then prone to polymerization and sickling crisis under conditions of low oxygen tension. One of the major causes of mortality in SCD is acute chest syndrome (ACS). On the other hand, coronavirus disease 2019 (COVID-19) is a pandemic disease that carries significant mortality and morbidity worldwide with unknown outcomes in the affected SCD population. This study was created for that reason. MATERIALS AND METHODS: We report a case series of ten SCD patients who were affected by COVID-19 and required admission between May 1, 2020, and October 30, 2020, at a tertiary care hospital in Dhahran, eastern region of Saudi Arabia. Historical data were obtained retrospectively from electronic records. MS Excel was used for data entry, and SPSS version 23 was used for data analysis. RESULTS: The mean age of the patients involved in the study was 32 years, and the mean duration of symptoms was 5.7 days. None required critical care admission, and there was no mortality. All patients were discharged from hospital in good condition with no requirement of home oxygen. CONCLUSION: Although we expected a fatal outcome of SCD patients affected by COVID-19 infection, our limited case series showed favorable disease behavior and outcome, with a suspicion of underlying unclear protective mechanism from serious complications. However, further studies are required to better understand COVID-19 behavior in SCD patients.

Remsima (a Tumor Necrosis Factor (TNF) -α Inhibitor) induced hemolysis in a patient with Crohn's disease - Case report.

INTRODUCTION: Crohn's disease (CD) is an idiopathic inflammatory disorder of unknown etiology with genetic, immunologic, and environmental influences. Infliximab is a treatment modality for fistulated Crohn's disease. Infliximab induced hemolysis is rare and very few cases reported before in Ulcerative colitis (UC) but not in Crohn's disease. CASE PRESENTATION: We are reporting a 63 years old gentleman who was diagnosed as Crohn's disease and started on Tumor necrosis factor Inhibitor (TNF) -α i.e. (infliximab - Remsima) infusion. The course was complicated by Coomb's negative hemolytic anemia which is suggestive of non-immune drug induced hemolysis. Our patient was treated with steroid and conservative measures. Upon following up, his hemoglobin level as well as all hemolytic markers showed dramatic improvement. Adalimumab was used to treat this patient as an alternative choice without relapse of hemolysis. CLINICAL DISCUSSION: Drug induced Hemolysis is not a well-known complication post receiving Tumor necrosis factor Inhibitor (TNF) -α infusion in patients with Crohn's disease. Coombs negative hemolysis keeps in favor of non-immune drug induced rather than other differentials in our case scenario. CONCLUSION: Although cross-reactivity is expected between one biological agent to another, in our case the use of Adalimumab as alternative choice post Tumor necrosis factor Inhibitor (TNF) -α (Remsima infliximab) induced hemolysis did not cause hemolysis or any type of reaction.

Isolated ten-eleven translocation 2 positive in triple negative essential thrombocythemia: Case report and literature review.

Essential thrombocythemia is one of the famous diseases under the category of myeloproliferative disorder. It is an end result of a genetic mutation of one or more of the most frequent oncogenes such as Janos kinase 2 (JAK2), MPL proto-oncogene, thrombopoietin receptor (MPL), and calreticulin (CALR). However, negative genetic markers, so-called (triple negative disease), can happen in the presence of other uncommon types of mutation. TET2 (ten-eleven translocation 2) positive as isolated genetic marker in triple negative essential thrombocythemia is uncommon genetic presentation. For that, we are reporting a 22-year-old lady who presented with a feature of dyspepsia and accidentally found to have persistently high platelet count, even after treating her mild iron deficiency anemia with no other secondary causes. Further investigations and bone marrow biopsy supported the diagnosis of isolated TET2 positive in triple negative essential thrombocythemia. We treated her conservatively with good hydration and low dose of aspirin. In conclusion, isolated TET2 positive in triple negative essential thrombocythemia at presentation is uncommon with no clear management or risk stratification guideline. However, it is hypothesized that TET2 mutation precedes JAK2; therefore, the detection of isolated TET2 in a triple negative essential thrombocythemia case should be closely followed for clonal evolution in long term. Further study and guidelines required in this area.

Chronic Neutrophilic Leukemia with Monocytosis.

Chronic neutrophilic leukaemia is a very rare disease with diagnosis based on persistent leucocytosis >25×103/µl and monocytes <1×103/µl. The revised WHO criteria 2016 included CSF3R gene mutations as a diagnostic finding. We report the case of a 77-year-old man who was found to have asymptomatic persistent mature neutrophilic leucocytosis with monocytosis. Molecular study confirmed the presence of a CSF3R gene mutation in the absence of morphological or genetic features of myelodysplasia or other forms of myelodysplastic syndrome. The patient's medical history was significant for coronary artery disease, hypertension, chronic obstructive pulmonary disease, bilateral cystic bronchiectasis, moderate pulmonary hypertension, tuberculosis treated 27 years previously, hypothyroidism, and a thyroid nodule. He had hepatosplenomegaly but no lymphadenopathy, and no other malignancy was seen on computed tomography (CT) scanning. At the time of evaluation, he was free of symptoms and had no evidence of infection or drug-induced leucocytosis. The patient was referred to an oncology centre and treated with hydroxyurea and subsequently azacitidine. However, he developed pancytopenia with bone marrow aplasia. He died with neutropenia sepsis. The presence of persistent monocytosis in this case created a diagnostic dilemma as to whether the disease was a variant of chronic neutrophilic leukaemia or was reactive monocytosis. LEARNING POINTS: The presence of a CSF3R gene mutation is diagnostic for chronic neutrophilic leukaemia (CNL).The monocytosis in this patient might have been a new variant of CNL.

ABO in Correlation to the Requirement of Mechanical Ventilation and Mortality in Critically Ill Patients With COVID-19.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that was first discovered in December 2019 and turned to be pandemic in early March 2020. We aimed to describe the dominant ABO group and outcomes of critically ill patients (respiratory failure requiring mechanical ventilation and mortality) in a Saudi Arabian setting. METHODS: We conducted an observational, analytic cross-sectional, retrospective study in a tertiary care hospital. Around 90 candidates tested positive for COVID-19 were enrolled in this study during admission to critical care unit between May 2020 and September 2020. Blood group was detected in all patients included in the study during admission to critical care unit. RESULTS: In this study, data of 90 patients with COVID-19 admitted to critical care unit were collected. Some prevalent medical conditions were collected, in which hypertension (64.2%) and diabetes mellitus (58.9%) were the most reported comorbidities among patients and there was no significant difference between groups. Most of the sample had blood group of O (45.6%), while the least group was AB (5.6%). Patients with blood group of A/AB showed the highest mortality vs. group O/B (32% vs. 18.5%) with significant P value of 0.001. Patients of groups A/AB had higher risk for intubation than O/B groups (52.0% and 49.2%, respectively; confidence interval of 0.44 - 2.8 with insignificant P value of 0.055). Length of stay in critical care unit was significantly higher in group A/AB with a mean course of 18.20 days in comparison to group O/B with a mean course of 12.63 days (P = 0.033). CONCLUSION: Our data indicate that critically ill patients with COVID-19 with blood group A/AB are at increased risk of mortality and length of stay in critical care unit, with insignificant requirement of mechanical ventilation when compared with patients with blood group O/B. Future larger studies are needed to validate and understand the underlying mechanisms.

Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease.

BACKGROUND: Sickle cell disease is a genetic condition frequently found in Africa and the Arabian Peninsula. Uncommon complications include subgaleal haematoma (soft head syndrome) and periorbital oedema. CASE PRESENTATION: A 17-year-old male patient presented with body aches and progressive right parieto-temporal and frontal head swelling. Physical examination revealed puffiness of the right eye that progressed rapidly to reddish periorbital oedema sparing the extraocular muscle and pupil response to light. CT and MRI of the brain suggested multiple subgaleal haematomas (soft head syndrome) and right periorbital oedema. CONCLUSION: Subgaleal haematoma (soft head syndrome) and periorbital oedema are uncommon complications of sickle cell disease. Management is conservative rather than surgical. LEARNING POINTS: Subgaleal haematoma concurrently with periorbital oedema is a rare presentation of sickle cell disease.There are no guidelines on treatment, but the conditions in our patient resolved with conservative management.

Critical Care COVID-19 Patient with a Picture of Thrombotic Thrombocytopenic Purpura.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is an uncommon haematological disease which can occur at any age and may present with COVID-19. This case describes a COVID-19 complication associated with a presentation resembling TTP. CASE DESCRIPTION: A 51-year-old man who had received a kidney transplant and was on immunosuppressant medication, was admitted to a critical care unit with severe COVID-19 pneumonia/acute respiratory distress syndrome (ARDS) which required intubation, mechanical ventilation and inotropic support. The course was complicated by the classic pentad of thrombocytopenia, intravascular haemolysis, acute kidney injury, neurological symptoms and fever, which prompted the diagnosis of probable TTP. After five sessions of therapeutic plasma exchange, the patient's general status improved, he was weaned off mechanical ventilation and his renal panel and haemolytic markers normalized. CONCLUSION: TTP is a life-threatening condition which requires urgent management with therapeutic plasma exchange. This case highlights some possible complications of COVID-19 generally and in immunocompromised patients specifically. The potential role of plasma exchange in COVID-19 patients without a positive diagnosis of TTP (the so-called 'TTP resembling presentation') is an area of further research. LEARNING POINTS: COVID-19 can manifest as a picture of thrombotic thrombocytopenic purpura (TTP) which requires therapeutic plasma exchange as in other cases of TTP.Further research is required on the use of therapeutic plasma exchange in severe COVID-19 with cytokine storm.