Pulmonary sarcoidosis with suspected bone marrow involvement presenting as severe thrombocytopenia: a case report.

Drug (vaccine)-induced thrombocytopenia 2021: Diversity of pathogenesis and clinical features.

A 62-year-old Caucasian female who had recently been diagnosed with multiple myeloma with a sacral plasmacytoma received induction treatment with bortezomib, cyclophosphamide, and dexamethasone. She had a significant response to therapy and had just begun the process of mobilization and collection of peripheral blood progenitor cells for autologous stem cell transplantation when she presented to the emergency center (EC) with pain and swelling of the left upper extremity. Her blood pressure on presentation was 114/69 mmHg with a heart rate of 98 beats per minute. Serum sodium was 136 mEq/L, potassium 4.3 mEq/L, chloride 98 mEq/L, carbon dioxide 24 mEq/L, blood urea nitrogen 9 mg/dL, serum creatinine 0.6 mg/dL, glucose 119 mg/dL, calcium 8.6 mg/dL, albumin 3.9 mg/dL, phosphorus 2.5 mg/dL, and magnesium 2.1 mg/dL. Liver function tests were within normal limits, with the exception of lactate dehydrogenase that was elevated at 728 IU/L. Prothrombin time (PT) was 16.5 sec (normal 12.7―15 sec), international normalized ratio (INR) 1.24, and activated PTT (aPTT) was 38.3 sec (normal 24.7―35.9 sec). D-dimer was >20 μg/mL (normal 0―0.4 μg/dL) and fibrinogen was 424 mg/dL. A complete blood count (CBC) showed a white blood cell (WBC) count of 11,400/μL, hemoglobin of 11.6 g/dL, and platelet count of 14,000/μL. Review of the peripheral smear revealed the presence of anisocytosis and ovalocytes, and the absence of fragmented red blood cells (schistocytes). The platelet count had dropped from 316,000/μL to 14,000/μL in a 7-day period (see Fig. 1). Home medications included zolpidem, lorazepam, duloxetine, famotidine, fentanyl, hydromorphone, and ondansetron. Doppler ultrasound study showed a completely occlusive thrombus within the left internal jugular vein extending to the subclavian, axillary, brachial, and basilic veins. The patient had recently had a left subclavian central venous catheter (CVC) placed 11 days prior to presenting to the EC. The CVC was removed, but no anticoagulation was started secondary to severe thrombocytopenia.

IntroductionSarcoidosis is an inflammatory disorder characterized by the presence of non-caseating granulomas in affected organs. The presence of CD4-positive T lymphocytes and macrophages in affected organs suggests an ongoing immune response. Systemic corticosteroids remain the mainstay of treatment, but therapy is often limited by adverse effects. This is the first report of the use of adalimumab (HUMIRA®, Abbott Laboratories, North Chicago, IL, USA), an anti-tumor necrosis factor monoclonal antibody, in a patient with systemic sarcoidosis with bone marrow involvement.Case presentationA 42-year-old African-American man with a medical history significant for hypertension and diabetes mellitus presented with anemia and thrombocytopenia of two months duration. The patient underwent physical examination, bone marrow aspiration and biopsy, chest X-ray, acid-fast bacilli stain, computed tomography with contrast, and additional laboratory tests. He was diagnosed with systemic sarcoidosis with splenomegaly and bone marrow involvement. Drug therapy included prednisone, which had to be discontinued owing to adverse effects, and adalimumab.ConclusionThis is the first report describing the use of adalimumab in a patient with systemic sarcoidosis with bone marrow involvement. Tumor necrosis factor antagonism with adalimumab was efficacious and well-tolerated in this patient and may be considered as a treatment option for similar cases.

Most of the newly diagnosed cases of thrombocytopenia in pregnancy are mild, asymptomatic and accidentally discovered on routine antenatal screening. Common causes for the same include gestational thrombocytopenia, Preeclampsia, HELLP syndrome, and less commonly immune causes like ITP. As a matter of fact, HELLP and Preeclampsia have specific diagnostic signs and symptomatology, others are rather difficult to distinguish, as they are usually asymptomatic. We present a case of 21 years second gravida at 35 weeks and six days period of gestation, referred from a local practitioner for severe thrombocytopenia (Platelet count-20000/mm3). She had history of previous still birth due to cord prolapse, and severe thrombocytopenia (Platelet count-6000/mm3) in previous pregnancy. She recovered rapidly and spontaneously in postpartum period. The newborn platelet count was also normal. She was considered to be a case of rare but severe recurrent gestational thrombocytopenia, after ruling out other causes of severe thrombocytopenia in pregnancy. Management includes adequate preparations for ensuring optimal fetomaternal outcome. Treatment initiation and modification should be done, with preparation and anticipation for regional anesthesia, blood loss and appropriate styptic measures.

Background Thrombocytopenia is defined as a plate-let count 72 hours)-onset thrombocy-topenia are caused by different categories of underlying conditions. Chronic fetal hypoxia and sepsis or necrotiz-ing enterocolitis are by far the most frequent causes of, respectively, early-and late-onset neonatal thrombocy-topenia.1,3 Methods We describe the clinical case of a SGA (small for gestational age) neonate who experienced a severe ranitidine-induced thrombocytopenia. An extensive lit-erature search was performed to document other cases of ranitidine-and H2-antagonist-induced thrombocyto-penia. Furthermore, other case reports of drug-induced thrombocytopenia in newborns were explored. Results We report on a late preterm male infant, who showed an unexpected, severe thrombocytopenia (8 × 109/L) at day 5 of life. He was born SGA and had also showed a mild early-onset thrombocytopenia with a low-est platelet count of 87 × 109/L on day 1, spontaneously normalising by day 3 (169 × 109/L). The low platelet count on day 5 only minimally responded to platelet transfu-sion. It did however recover completely within 5 days after cessation of ranitidine (4 × 0.5 mg/kg/day), which was started on day 3 of life in a context of feeding diffi-culties. Other causes of neonatal thrombocytopenia were explored and ruled out. The likelihood of an adverse drug reaction in this case was indicated as ‘probable’ on the Naranjo scale.4 Besides a brief report on a cimetidine-in-duced thrombocytopenia over 25 years ago,5 no other neonatal or paediatric cases of H2-antagonist-induced thrombocytopenia have been reported to date. Several adult cases have been published nevertheless.6 It seems that neonatalthrombocytopenia, although one of the most frequent haematological conditions in newborns, is only rarely attributed to an adverse drug reaction. Conclusion Neonatal thrombocytopenia is a frequent haematological abnormality and has a variety of causes. In rare cases, this low platelet count might be caused by an adverse drug reaction, supposedly immune-mediated. Although H2-antagonist are widely used in paediatric and neonatology departments, we describe the first case of a severe ranitidine-induced thrombocytopenia in a neo-nate. We believe that SGA infants are more at risk because of their inherent state of bone marrow depression at birth. Clinicians should be aware of the risks for (unexpected) adverse reactions, especially in routinely used drugs and in critically ill patients. Case reports may aid in expanding our knowledge of rare pharmacological complications.

Thrombocytopenia, one of many immune-related adverse events (irAEs), is a rare entity about which little is known on its treatment, outcomes, and patient demographics. Herein we present a case of severe thrombocytopenia after administration of pembrolizumab as an anti-programmed death-1 (PD-1) antibody. A 66-year-old man with advanced non-small cell lung cancer (NSCLC) received pembrolizumab; 21 days later, his platelet count was progressively decreased and he experienced severe thrombocytopenia (grade 4; platelet count 0.4×109/l). With oral steroids 1 mg/kg/day, the platelet count improved sufficiently; thus, a definite diagnosis of severe irAE-related thrombocytopenia was performed. Several reports have described the management and occurrence of severe thrombocytopenia after immune checkpoint inhibitor administration in patients with different neoplasms. Physicians should be alert to the potential of rare irAEs, such as severe thrombocytopenia.

Several case reports deal with the relationship between hepatitis C virus (HCV) infection and pulmonary or hepatic sarcoidosis. Most publications describe interferon α-induced sarcoidosis. However, HCV infection per se is also suggested to cause sarcoidosis. The present case report describes a case of biopsy-verified lung and liver sarcoidosis and HCV infection, and the outcome of antiviral therapy. In March 2009, a 25-year-old man presented with moderately elevated liver enzymes without any clinical symptoms. The patient was positive for HCV antibodies and HCV RNA of genotype 1b. Four months later the patient became dyspnoic and pulmonary sarcoidosis was diagnosed by lung biopsy and radiography. A short course of corticosteroid treatment relieved symptoms. Three months later, liver biopsy showed noncaseating granulomas consisting of epithelioid histiocytes and giant cells with a small amount of peripheral lymphocyte infiltration, without any signs of fibrosis. Chronic HCV infection with coexistence of pulmonary and hepatic sarcoidosis was diagnosed. Antiviral therapy with peginterferon α and ribavirin at standard doses was started, which lasted 48 wk, and sustained viral response was achieved. A second liver biopsy showed disappearance of granulomas and chest radiography revealed normalization of mediastinal and perihilar glands. The hypothesis that HCV infection per se may have triggered systemic sarcoidosis was proposed. Successful treatment of HCV infection led to continuous remission of pulmonary and hepatic sarcoidosis. Further studies are required to understand the relationship between systemic sarcoidosis and HCV infection.

Avatrombopag Safe and Effective in Treatment of Severe Refractory Thrombocytopenia in a Pediatric Patient with Philadelphia Chromosome Positive Acute Lymphoblastic Leukemia Following Allogeneic Hematopoietic Stem Cell Transplantation

Blood product transfusions for pediatric Jehovah's witness patients: Management recommendations and considerations

Introduction Introduction/Background: PAP Intolerance is a common initial complaint among new PAP users, with mask issues and difficulty with pressure level leading the list. In clinical practice, feelings of claustrophobia and anxiety are usually cited as the reason for PAP intolerance. Sometimes craniofacial impairments such as congenital malformations or change in facial architecture after a major accident or surgery may play a role. Our case describes our experience with a patient afflicted with severe sinonasal cutaneous Sarcoidosis. Report of case(s) Case Description: A 43 year old AAF with a PMH of morbid obesity, depression and cutaneous sinonasal Sarcoidosis was referred to Sleep Medicine by ENT in late May 2020 with complaints of snoring, witnessed apneas, excessive daytime sleepiness, fatigue, and non-restorative sleep for >20 years. The patient was first diagnosed with cutaneous Sarcoidosis limited to the internal sinonasal region around 2015 and managed by ENT until the lesions spread to her face. Due to this, the patient developed depression and became increasingly non-complaint with her medical regimens. As a result, her sarcoidosis and other co-morbidities worsened. Diagnostic Polysomnogram (PSG) in May 2020 showed presence of Hypopnea predominant Obstructive Sleep Apnea (OSA) with AHI 35.9 and O2 desaturation to 58%. Subsequently, the patient was placed on auto CPAP 6–20 cm H2O with a DreamWear FFM. At follow up in September 2020, her sarcoidosis had spread to her upper lip area, so she was prescribed a Fit Life mask to avoid this region. Follow up in December 2020 revealed improvement in Sarcoidosis and accompanying vast improvement in objective compliance though she reverted to using her DreamWear FFM due to “mask seal issues. An AirFit f20 mask was then given to help avoid the active lesions on her upper lip. Conclusion: Discussion/Conclusion In patients with compromised facial architecture, special attention must be paid to the type of mask interface used so it does not aggravate sensitive areas which may lead to poor compliance with PAP therapy. Instructions were given for her to alternate between her three masks—the Fit Life, the DreamWear FFM and the AirFit f20 FFM—in an effort to reduce irritation on her face over time. Support (if any):

Immune Thrombocytopenia during Childhood: New Approaches to Classification and Management

To investigate the clinical characteristics of sarcoidosis with upper airway (including nose/paranasal sinus, pharynx, larynx and middle ear) involvement as the presenting symptoms, and therefore to minimize the misdiagnosis of sarcoidosis with special manifestations. Four cases of sarcoidosis with upper airway involvement as the presenting symptoms diagnosed at our hospital were described. The clinical data were analyzed and related literatures were reviewed. Three female patients aged 52, 53, 34 years and one 15-year-old male patient, with the main complaints as "mycteric mass", "chronic otitis media", "hoarseness" and "chronic tonsillitis" respectively, were referred to our hospital for further evaluation. The diagnosis of sarcoidosis was finally confirmed by biopsy of bronchial mucous membrane, enlarged peripheral lymph nodes, and otorhinolaryngological lesions. Physical examination and imaging findings showed involvements of the lungs and/or the lymph nodes in 3 patients. Seven Chinese articles about upper airway sarcoidosis (all involving the nose) were found after literature search with "sarcoidosis" as the key word at the CNKI database (1915 - 2011). English literature search with the same key word at "Pubmed" showed that the rate of upper airway involvement in sarcoidosis varied from 2.3% - 6.0%, mostly concurrent with thoracic and lymph node diseases, whereas cases of sarcoidosis presenting with otorhinolaryngological symptoms were occasionally reported. Although upper airway sarcoidosis was rare, it may be the cause of chronic otorhinolaryngological disease which responded poorly to routine treatments. Careful collection of medical history, physical examination and necessary accessory examinations, especially better understanding of the exceptional manifestations of sarcoidosis, can help to minimize misdiagnosis and therefore to improve the prognosis of the patients.

Background: Trastuzumab, a humanized monoclonal antibody directed against human epidermal growth factor receptor 2 (HER2), has been shown to be active against metastatic gastric cancers that overexpress HER2. Case Report: A 47-year-old man presented with a headache and visual disturbance. He was subsequently found to have an intracranial hemorrhage. Laboratory testing showed severe thrombocytopenia, and a bone marrow biopsy revealed aggregates of malignant tumor cells. Endoscopic biopsy of an ulcerative lesion of the gastric antrum confirmed signet ring cell carcinoma based on the results of H&E staining. Immunohistochemistry of the tumor cells revealed HER2 overexpression with an intensity of 3+, and silver in situ hybridization showed HER2 gene amplification. The patient was treated with trastuzumab because of the presence of severe thrombocytopenia. After 2 months of trastuzumab therapy, gastric wall thickening and ascites were diminished and thrombocytopenia was markedly improved. Trastuzumab was continued for an additional 3 months. Conclusion: This is the first report of a positive response to trastuzumab in a patient with HER2-overexpressing metastatic gastric cancer that was accompanied by bone marrow involvement and severe thrombocytopenia. This finding is of considerable relevance for difficult cases of metastatic gastric cancer that preclude the administration of aggressive antineoplastic regimens.

Cutaneous sarcoidosis may be a chronic disease with important morbidity requiring aggressive therapy. The efficacy of different anti-tumor necrosis factor α(anti-TNF-α) treatments in refractory cutaneous and systemic sarcoidosis has been reported previously. We report the first patient with chronic cutaneous sarcoidosis who responded to dose escalation of anti-TNF-α agents that have been ineffective at the standard dosage, illustrating that the optimal dosing regimen has still to be defined for this indication before considering difficult-to-treat patients as nonresponders. Our case report also illustrates that the fusion protein etanercept, even used at a high dosage, may be less effective for the treatment of cutaneous sarcoidosis than the monoclonal antibodies infliximab and adalimumab.

Systemic lupus erythematosus (SLE) is often seen with antiphospholipid antibody syndrome (APS), and these conditions may occur concurrently with severe immune thrombocytopenia (ITP) and even acute kidney injury (AKI); however, post-renal AKI due to bleeding is uncommon. Here, we describe a case of post-renal AKI and anuria in a patient with SLE and APS, which were attributable to urinary tract obstruction due to massive blood clots caused by secondary ITP. A 50-year-old Japanese woman was admitted to our hospital with anuria, abdominal tenderness, purpura in the trunk and in both legs, and severe thrombocytopenia. She had been receiving medical treatment for APS and SLE till the age of 45 years. Computed tomography revealed a blood clot without extravasation in both urinary tracts, and she was diagnosed with post-renal AKI due to complete obstruction of the urinary system. Additionally, based on her medical history, elevated platelet-associated Immunoglobulin G (IgG) levels, and increased megakaryocyte count, she was diagnosed with secondary ITP complicated by SLE and APS. She also had elevated APS-related autoantibodies, including antiphosphatidylserine/prothrombin Immunoglobulin M (IgM), and IgG. However, concomitant serositis such as lupus enteritis or cystitis was not seen. She was treated with a combination of glucocorticoids, intravenous immunoglobulin, and continuous haemodialysis/haemofiltration, which resulted in rapid improvement of her symptoms and renal dysfunction. Secondary ITP-induced massive bleeding of urinary tract can cause post-renal AKI. Appropriate diagnosis and aggressive treatment are necessary to improve prognosis in such patients.