BACKGROUND: Hypothyroidism is associated with a shift of the hemostatic system to a hypocoagulable state and inhibited platelet function. However, previous studies did not consider the different degrees of response to thyroxine treatment and its effect on platelet function. This raises the need for further studies to clarify the effect of different degrees of responses to treatment on platelet function in hypothyroid disease. OBJECTIVES: To characterize the abnormalities in the primary hemostasis (platelet adhesion and aggregation) in patients with overt hypothyroidism on L-thyroxine therapy and to study the effect of variation of the responses to treatment on platelet function. MATERIALS AND METHODS: This cross-sectional study includes 64 patients with overt hypothyroidism on L-Thyroxine treatment. The patients were divided into three groups according to their response to treatment. Group-I: euthyroid patients (ET) (n = 25): (normal thyroid-stimulating hormone [TSH] and free T4), Group II: subclinical hypothyroid (SH) (n = 22): (high TSH and normal free T4), and Group III: inadequately treated hypothyroid (IH) (n = 17) (high TSH and low T4). Platelet function was assessed by light transmissiom aggregation response to adenosine diphosphate (ADP), arachidonic acid (AA), epinephrine (EPN), collagen, and ristocetin and by the platelet function analyzer (PFA-100) closure times (CTs). RESULTS: Platelet aggregation responses showed a significant reduction to ADP in ET patients (42.9 ± 20.7), SH patients (41.8 ± 24.2), and IH patients (46.9 ± 20.1) and to risocetin in ET patients (62.9 ± 22.8), SH patients (62.6 ± 21.2), and IH patients (61.3 ± 14.6) as compared to the controls (59.1 ± 9.5) and (75.3 ± 6.9), respectively. There is a significant prolongation of C/EPN: (175.6 ± 82.5) in the IH patients as compared to the controls (141.6 ± 26.8). Significant prolongation of C/ADP CT (132.2 ± 72.5) in IH patients as compared to the controls (100.7 ± 24.1) was found with normalization of both CT in ET patients. CONCLUSIONS: In overt hypothyroidism using two different tests of platelet function, we confirmed that the existence of a hypocoagulable state is due in part to a defect in primary hemostasis. Moreover, the defect varies according to the degree of response to L-Thyroxine treatment; it is more pronounced in inadequately treated hypothyroid patients and less in the subclinical hypothyroid.

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A 38-year-old man presented with mild fatigue of recent onset to the medical outpatient department (OPD). Clinical examination showed an absence of fever, pallor, and no organomegaly. Routine complete blood count (CBC) showed mild leukocytosis (total leukocyte count 22.93 × 10⁹/L), absence of features of sepsis but with immature granulocyte (IG) fraction of 26.6% on peripheral blood film without basophilia or eosinophilia. A diagnosis of chronic myeloid leukemia (CML) was suspected which was confirmed by conventional karyotyping and quantitative reverse transcription − polymerase chain reaction (QPCR). Following administration of imatinib for 2 weeks, a follow-up CBC showed a reduction in IG fraction to 1.5%. The patient is under follow-up in OPD for 4 months and is responding well as substantiated by follow-up QPCR. To the best of our knowledge, this is the first ever report where CML was first suspected on the basis of IG fraction and the same parameter proved useful for follow-up.

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BACKGROUND: Sickle cell disease (SCD) is one of the common hereditary blood diseases in Saudi Arabia. The hepatobiliary system is one of the common organs to be affected either directly from the sickling process or indirectly as a result of chronic hemolysis and multiple blood transfusions. Cholelithiasis is one of the common complications of sickle cell anemia. OBJECTIVES: To assess the prevalence of cholelithiasis in children with SCD treated at King Saud Medical City (KSMC); Pediatric Hospital in Saudi Arabia and describe the outcome, management, and clinical profile of children with SCD and cholelithiasis. MATERIALS AND METHODS: This is a retrospective descriptive study conducted at a single tertiary health care center, KSMC, Pediatric Hospital, Riyadh from 2012 to 2019 on 277 patients aged <14 years. Medical records of all pediatric patients with SCD included in the study were identified and reviewed. RESULTS: From total of 277 patients, 87 (31.4%) had cholelithiasis. Forty three (49.4%) of them were female and 44 (50.6%) were male. From 87 patients who developed cholelithiasis, 15 patients aged <5 years old, 50 aged (5–10 years), and 22 aged >10 years. According to the genotype of sickle cell (SS), the occurrence of cholelithiasis were (75.9% in SS, 23% in S/BETA 0 and 1.1% in S/BETA+). The majority were symptomatic (59.8%) compared to asymptomatic (40.2%) and 26 patients (29.9%) were complicated. In our study, we found that complications for the gallstones were acute calcular cholecystitis, chronic cholecystitis, direct hyperbilirubinemia, transaminitis, and acute pancreatitis. CONCLUSION: The prevalence of cholelithiasis in our study was significant. The large majority of patients were symptomatic. Cholecystectomy must be strongly recommended in symptomatic patients.

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Diarrhea-associated hemolytic uremic syndrome (HUS) occurs sporadically in the community. It is well known in the pediatric age group but also occurs in the adults, albeit with lower frequency. Prompt recognition of diarrhea-associated HUS is important, as it can be easily overlooked, as prerenal acute kidney injury. Besides this, management of diarrhea-associated HUS is different; varying from supportive care with meticulous observation for spontaneous recovery to occur, or to institute specific therapy such as plasmapheresis or immunosuppressant drugs, if it evolves into organ or life-threatening thrombotic microangiopathy (TMA). HUS is one type of TMA. Steroids have been used anecdotally either standalone or in combination with other therapies, in the management of TMA, especially if it is immune-mediated TMA. In our two cases of diarrhea associated TMA/HUS, we used steroids successfully as an empiric therapy for life threatening TMA.

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BACKGROUND: Diffuse large B-cell lymphoma (DLBCL) is representing 30%–40% of all lymphomas. It is an aggressive lymphoma with heterogeneous clinicopathological features. Inflammatory processes have been identified to play an important role in the pathogenesis of lymphoma including the neutrophil-lymphocyte ratio (NL ratio) was associated with a poor prognosis. AIM OF STUDY: The aim of this study is to assess neutrophil/lymphocyte (N/L) ratio in relation to clinical presentation, and other prognosticators in DLBCL, and to study the effect of these markers with response rate and early outcome. PATIENTS AND METHODS: This is a cohort prospective study with data obtained from May 2018 to November 2019. Data collected from multiple hematological centers in Baghdad, Iraq. A total of 58 adult patients who are newly diagnosed with DLBCL were enrolled. In addition to demographic features, international prognostic index (IPI) score, complete blood parameters (white blood cell count, N/L ratio, had assessed. RESULTS: The mean age was 53.54 ± 14.95 years. Twenty-two (45.83%) had extranodal involvement at the presentation. Advanced stage was reported in 34 (70.83%). Median N/L ratio was 3.39 with cut-off values 4.41. There was no significant association with the N/L ratio neither with progression-free survival (PFS) nor with the advanced stage presentation (P = 0.238, 0.343, respectively). It is found also that higher median N/L ratio was significantly associated with high and high-intermediate IPI score (P = 0.03). CONCLUSION: The NL ratio has a significant association with the IPI score, but not with the disease PFS.

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BACKGROUND: Evidences indicate that COVID-19 infection causes hypercoagulable state with micro and macrovascular thrombosis. Platelet-derived microparticles (PDMPs) have inflammatory and diverse coagulant roles. AIM: The aim of the study was to assess PDMPs in patients with active and convalescent post COVID-19 infection and correlate PDMPs with clinical, radiological and laboratory findings used in follow up. PATIENTS AND METHODS: The study enrolled 25 patients during active COVID-19 (Group A), of them five patients had thromboembolic events (TEE); and Group B including 32 patients during post COVID-19 stages. Clinical and radiological assessment, routine biomarkers, and detection of PDMPs levels, using enzyme-linked immunosorbent assay method, were done for all patients. RESULTS: In addition to significant differences detected regarding hemoglobin level, total leukocyte count, absolute neutrophil count, absolute lymphocyte count, C-reactive protein level, D-dimer, and serum ferritin, and high significant differences in PDMPs levels were elicited between groups A and B (mean ± standard deviation: 38.7 ± 10.6 IU/mL, and 18.9 ± 15.3 IU/mL) respectively, with discriminative level at 20.5 IU/mL. PDMPs showed nonsignificant difference between patients with and without TEE and no correlation was detected between PDMPs and clinical or radiological severity in post-COVID-19 patients. CONCLUSION: In COVID-19 infection, PDMPs are related to viral activity, and their major role is inflammatory associated.

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Autoimmune hemolytic anemia is a rare disorder with varied presentations. A primary physician could misdiagnose this condition for other simpler causes of anemia, if not aware of the spectrum of the signs and symptoms of this disease. This may lead to further worsening of the patient, due to delay in starting of the therapy with immunosuppresants. Hasty blood transfusion could cause exacerbation of hemolysis. Both IgG and IgM antibodies could cause this disease, and they are termed as warm and cold antibody hemolytic anemia, respectively. Monitoring and follow-up of patients are also very necessary along with careful tapering of the medications. Evolution of other autoimmune disorders such as systemic lupus erythematosus is also noticed in such children. There is a paucity of literature about this disease, especially from the developing world. In this study, we have shown the clinical profile of 21 children with autoimmune hemolytic anemia, with the treatment given and the response. An attempt to compare our data with the available data from various studies has also been made so that a primary care physician could easily identify the most common symptoms and signs of this disease and treat such children.

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Langerhans Cell Histiocytosis (LCH) in adults is rare and has little prospective data to guide therapy. We describe a 36 year old gentleman who presented with a three-month history of isolated cervical lymphadenopathy, from which excision biopsy revealed sheets of large, atypical cells 10-15 um in size, with indented nuclei and fine chromatin along with eosinophilic microabscesses. Immunohistochemistry was positive for CD3, CD20, CD68, CD1a, S100, CD4, Langerin (CD207) and CD45 with a Ki67 index of 10%. PET scan showed FDG-avid uptake in left mastoid air cells, which was confirmed by MRI to indicate bony involvement with disease. Bone marrow and CSF were normal. He was diagnosed as multisystem LCH without risk organ involvement and initiated on single agent cytarabine at 100 mg/m2 five days a month. A PET scan after three cycles showed near complete metabolic resolution. He has completed six cycles of the same and is asymptomatic. He is planned for continuation of Cytarabine for another of 12 to 14 months. Single agent cytarabine appears safe and effective for adult patients with LCH. We provide a short summary of available data: compilation of prospective data will further clarify the utility of this regimen in adult patients with LCH.

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Extramedullary involvement in multiple myeloma (MM) is seen in 7%–18% of cases. The common organs involved are skin and upper respiratory tract. The uncommon organs involved include the liver, spleen, kidney, pleura, lymph nodes, and soft tissue. Central nervous system (CNS) involvement is extremely rare and occurs in only about 1% of patients. We present an interesting case of nonsecretory MM (NSMM) with atypical involvement of the liver, spleen and CNS. An elderly female patient initially presented with low backache. Skeletal survey showed multiple lytic bony lesions. An initial diagnosis of plasma cell neoplasm was made based on the biopsy of the sacral lytic lesion. No monoclonal gammopathy was found in the serum or urine electrophoresis. A diagnosis of NSMM was made and the patient was started on bortezomib, dexamethasone, lenalidomide (VRD regime). However, over the next 2 months or so, the patient was found to have involvement of liver, spleen, and meninges on imaging despite chemotherapy. The plasmablastic lesions were confirmed on liver biopsy, bone marrow, and cerebral spinal fluid study. Patient showed remarkable clinical improvement on addition of daratumumab to the VRD regime and is currently under maintenance therapy. Repeat imaging shows the reduction in lytic lesions. This case is reported as a rare combination of NSMM with CNS involvement.

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The outbreak of the novel coronavirus-19 (COVID-19) rapidly grew into a worldwide pandemic. The link between the disease severity and higher levels of inflammatory markers was reported including cases of hemophagocytic lymphohistiocytosis (HLH), a potentially life-threatening disorder. We report herein a case of HLH trigged by COVID-19 infection and we review all reported cases of HLH secondary to COVID-19 among immunocompromised patients by searching PubMed publications till July 2021. A 69-year-old woman with a previous medical history of diabetes mellitus and rheumatoid arthritis treated with oral steroids presented for a 5-day history of fever, persistent cough, anorexia, and dyspnea. The diagnosis of COVID-19 was confirmed. She received empiric antibiotic therapy, oxygen supply, and corticosteroids. On day 17, laboratory investigations revealed bicytopenia with a platelets rate of 31,000/mm³ and an hemoglobin rate of 8.2 g/dL. Hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia were noted. Bone marrow aspiration and biopsy revealed images of hemophagocytosis. The HScore yielded 200 points, representing 80%–88% probability of HLH. We continued corticosteroids and treatment. The disease evolution was favorable. The diagnosis of HLH secondary to COVID-19 shoud be considered in front of cytopenia, hyperinflammatory state, and a worsening clinical condition. Prompt diagnosis and treatment improve the prognosis.

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To highlight the graveness of invasive Aspergillus infections that could end in serious complications in immunocompromised patients, we are going to report a rare case of bilateral Aspergillus endophthalmitis in a lymphoma patient. We present a case in an academic referring hospital. The patient was an immunocompromised patient with diffuse large B-cell lymphoma complicated with vision loss due to Aspergillus infection diagnosed with B-mode ultrasound and culture and followed by treatment with vitrectomy and intravitreous amphotericin. The patient was followed for 9 months, culminating in visual acuity of 4/100 and 1/100 in her left and right eye, respectively. Despite appropriate diagnosis and treatment, invasive Aspergillus infection may still have a poor prognosis.

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