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Unusual presentations of sarcoidosis pose a diagnostic challenge and warrant attention. Hematologic associations: Case 1 (37-years-old male): Pancytopenia: myelofibrosis (leading to sepsis and mortality) following a two-year quiescent course of biopsy-proven-sarcoidosis. Case 2: (38-years-old male): Presentation with thrombocytopenia (5 × 10³/cmm):...
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Context 1
... infections and laboratory investigations revealed rapidly progressive pancytopenia with no history of new drug intake, and azathioprine was ceased. Examination at the time revealed no hepatosplenomegaly or lymph node enlargement. Laboratory investigations at the time are shown in Table 1. Bone marrow biopsy revealed evidence of myelofibrosis (Fig. 3), with immunohistochemistry showing normal CD34 pattern, occasional scattered CD20 positive cells, marked increase in CD68 positive macrophages, reticulin showing heterogeneous areas of fibrosis grade III, cytokeratin was negative which excluded secondary epithelial tumors, myeloperoxidase was positive with normal distribution, and ...
Citations
... Despite adequate treatment, however, greater than 10% of patients with pulmonary sarcoidosis develop progressive disease, and the disease itself carries a mortality rate of approximately 7% within a 5-year follow-up period. 4 Sarcoidosis should be on the differential in patients who present with persistent leukocytosis and multiorgan involvement, as in the patient discussed above. Multiorgan involvement can enhance the diagnostic certainty but only biopsy showing non-caseating granulomas confirms the diagnosis. ...
This paper summarizes the case of a patient admitted to the hospital for nausea, light-headedness, and vomiting in the setting of persistent leukocytosis. Despite being adequately treated for an infection, the patient’s leukocytosis did not resolve. Workup yielded a diagnosis of sarcoidosis. This is an unusual presentation of sarcoidosis since the patient did not present with typical respiratory symptoms. The presenting dyspnea on exertion is thought to be due to his smoking history. Treatment is promptly initiated with steroids. It is of utmost importance to initiate steroid therapy to prevent the progression of the disease. Sequelae may include glaucoma, small-fiber neuropathy, and pain syndromes.
... In a univariate regression, the only variable that was associated with the diagnosis of migraine was the female sex (OR: 2.7; 95% CI: 1.02-6.84). A few case reports have described patients with sarcoidosis and a migraine-like headache [35], while in other cases, the description of the headache does not allow its classification [36][37][38][39]. Sarcoidosis could be another comorbidity of migraine, since its frequency seems to be twice as high as what is expected to occur by chance [11]. ...
Citation: Tana, C.; Azorin, D.G.; Cinetto, F.; Mantini, C.; Tana, M.; Caulo, M.; Ricci, F.; Martelletti, P.; Cipollone, F.; Giamberardino, M.A. Common Clinical and Molecular Pathways between Migraine and Sarcoidosis. Int. J. Mol. Sci. 2023, 24, 8304. https://doi.; Tel./Fax: +39-871-357905 † The authors contributed equally to this work. Abstract: Migraine and sarcoidosis are two distinct medical conditions that may have some common biological and clinical pathways. Sarcoidosis is a chronic granulomatous disease characterized by the formation of granulomas in various organs, including the lungs, skin, cardiovascular system, lymph nodes, and brain. Migraine is a common comorbidity in sarcoidosis patients and a common neurological disorder characterized by recurrent headaches that can be accompanied by other symptoms , such as nausea, vomiting, and sensitivity to light and sound. There have been several reports of individuals with neurosarcoidosis experiencing migraines, though the exact relationship between the two disorders is not well understood. Both conditions have been associated with inflammation and the activation of the immune system. In sarcoidosis, the formation of granulomas is thought to be an immune response to the presence of an unknown antigen. Similarly, the pain and other symptoms associated with migraines are thought to be caused by inflammation in the brain and the surrounding blood vessels. There is also evidence to suggest an interplay of environmental and genetic factors playing a role in both conditions, but evidence is inconsistent with the hypothesis of shared genetic susceptibility. This review aims to illustrate common clinical and biological pathways between migraine and sarcoidosis, including inflammation and dysregulation of the immune system, with a focus on the cumulative burden of concurrent disorders and therapeutic implications.
... Further on CBC, anemia and leukopenia is seen in bone marrow involvement. 11,12 In our case, the patient had normal cellularity of the bone marrow with sufficient number of megakaryocytes and the absence of anemia and leukopenia, signifying no bone marrow involvement. Furthermore, our patient's history of fever, rhinorrhea, and green-colored sputum, for which he received amoxicillin 2000 mg for 6 days, could have triggered drug-induced thrombocytopenia. ...
Thrombocytopenia is a problem causing drop in platelet counts through different mechanisms. Patients typically present with petechiae to systemic bleeding, which are indications of a low platelet count. A smaller percentage of drop may be asymptomatic. The major mechanisms include increased sequestration in the spleen, underproduction from the bone marrow, and peripheral destruction. Many etiological factors can cause this. Sarcoidosis is one of the most uncommon etiologies. A careful diagnosis is required because, if the condition is not treated, it can be fatal. Steroid therapy and platelet transfusions remain the mainstay of treatment. Here, we describe a patient who presented with signs and symptoms of severe thrombocytopenia, which further led to the diagnosis of sarcoidosis. An adult male presented to the emergency department with a rash that deteriorated throughout the day. Based on complaints and laboratory testing, severe thrombocytopenia was noted. After a detailed examination and history-taking, he was found to have sarcoidosis. In association with drug administration and sarcoidosis, this could have caused severe thrombocytopenia. Written consent was taken from the patient mentioned in the study. The study was approved by the hospital and institutional ethics committee. Thrombocytopenia is a disorder where platelet counts drop below 150×109/l due to many different mechanisms. Among different etiological factors, sarcoidosis is the rarest and may present with very severe thrombocytopenia and lead to fatal complications. Such patients require close monitoring and treatment. Corticosteroids and platelet transfusions can be used as treatments. In the presented case, the patient was successfully treated, and on subsequent follow-up, the patient’s condition improved. Manifestations of severe thrombocytopenia can be present even before the diagnosis of sarcoidosis. Further, the history of amoxicillin administration due to infection could have triggered the appearance of thrombocytopenia. Confirmation of sarcoidosis was made via biopsy. Multiple etiological factors that resulted in diagnostic ambiguity in our patient's presentation include the diagnosis of sarcoidosis, a history of infection, amoxicillin, and mild splenomegaly. The patient's treatment and recovery may indicate that corticosteroids, in conjunction with platelet transfusions, are beneficial. This is a novel case report of the presentation of severe thrombocytopenia, which was present even before the diagnosis of sarcoidosis.
... Three articles were excluded, published between 1970 and 1980, due to difficulty finding the fulltext and only partial clinical information. Finally, twenty-one articles were selected [8,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30]. Two articles conducted in the same center had only partial information regarding patients with BFP and, therefore, could not be included in the final results [31,32]. ...
... Three articles were excluded, published between 1970 and 1980, due to difficulty finding the full-text and only partial clinical information. Finally, twenty-one articles were selected [8,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30]. Two articles conducted in the same center had only partial information regarding patients with BFP and, therefore, could not be included in the final results [31,32]. ...
Unilateral facial nerve palsy (FNP) is one of the most common cranial mononeuropathies. Among rare etiologies, neurosarcoidosis (NS) can cause bilateral involvement (both recurring and simultaneous) only in 15% to 25% of cases. The rarity of this systemic disease and its clinical heterogeneity, due to granulomatous inflammation that may affect many anatomic substrates, frequently make the diagnosis a real challenge for the clinician. Based on laboratory and instrumental tests, a careful diagnostic algorithm must be adopted to avoid misdiagnosis and delay in treatment. We present a 52-year-old woman with an acute onset of unilateral right FNP, rapidly developing contralateral involvement (simultaneous bilateral FNP). Lung findings pointed towards a systemic disease, and then lymph node biopsy confirmed NS. Corticosteroid therapy was started. After three years of follow-up, the patient is still in remission with a low prednisone dose. We discuss the differential diagnosis of bilateral FNP, focusing on clinical presentation, diagnosis, and treatment of NS. We have performed a literature revision, confirming bilateral FNP, outside Heerfordt syndrome, to be rare and sometimes represent the only neurological manifestation of NS onset.
The patient is a one-year-old girl referred to the hospital for an enlarged head after a 1.5-month history of two falls, followed by polydipsia, polyuria, and slow movement and growth. Three subsequent magnetic resonance imaging (MRI) examinations of the brain revealed nodular lesions disseminated in the brain parenchyma and intraventricular ependyma, resulting in obstructive hydrocephalus. Thoracic and abdominopelvic sonography showed no additional lesions. The preliminary diagnosis was a primary or metastatic neoplasm or infection. A biopsy of a lesion in the right frontal lobe was taken. The histological examination revealed features of Rosai-Dorfman disease (RDD), consisting of limited perivascular lymphoplasma cell infiltration with intervening sheets of proliferated histiocytes, with some of the histiocytes showing endocytosis of a single intact lymphocyte (emperipolesis).
Introduction:
We present an updated overview of the hematological involvementassociated with sarcoidosis, including a management approach forcytopenias and revisiting the association with hematologicalmalignancies.
Areas covered:
Theetiology of cytopenias in sarcoidosis can be attributed to two majoretiopathogenic mechanisms: infiltration of hematopoietic organs suchas the spleen and bone marrow, and autoimmune-mediated cytopenias.With respect to the association with hematological malignancies, itrequires careful evaluation of patients from a chronologicalperspective. Patients must be classified into one of three pathogenicscenarios, including preexisting hematological malignancies,synchronous development of malignancy and sarcoidosis due to commonpredisposing factors, or sarcoidosis as a predisposing factor formalignancies.
Expert opinion:
The association between sarcoidosis and hematologic involvement isbest understood as a pathogenic continuum, with cytopenias andhematologic neoplasms intertwined due to various etiopathogenicmechanisms. These mechanisms include sarcoid infiltration ofhematopoietic organs, common predisposing immunogenetics for thedevelopment of autoimmune cytopenias and malignancies, and anincreased risk of neoplasm development in patients with autoimmunecytopenias. Collaboration among the main specialties involved in theclinical management of these patients is crucial for an earlymonitoring and management.
Acute kidney injury (AKI) is one of the most important complications of critical illness and a significant public health concern. AKI is commonly associated with sepsis, cardiac dysfunction, and exposure to nephrotoxic medication; however, less common causes of AKI can lead to devastating patient outcomes when the underlying diagnosis is missed or delayed. These uncommon causes of AKI fall into 3 large categories: structural, immune mediated, and microvascular, including various types of thrombotic microangiopathy. Kidney imaging, urine studies, and serum hemolytic studies should be a routine part of the evaluation of AKI among critically ill patients.
Introduction
Sarcoidosis is a chronic granulomatous disease of unknown etiology. A variety of studies have pointed out that almost every part of the body can be affected, but it most often affected the lungs and intrathoracic lymph nodes. However, cases of sarcoidosis involving multiple organs in one patient are rarely reported. We describe a unique case of sarcoidosis, which was characterized by multiorgan involvement, including leg ulcers, splenomegaly, pancytopenia, and polyserositis. Glucocorticoids were effective during the treatment of the above lesions. This case highlights the diversity of clinical manifestations of sarcoidosis and emphasizes the importance of its differential diagnosis and the periodical follow-up. These are crucial to physicians in the diagnosis and treatment of sarcoidosis.
Main Symptoms and Important Clinical Findings
A 30-year-old male complained about intermittent fever 3 years ago. A computed tomographic scan of the chest showed lymphadenopathy in the mediastinum and hilar regions. Routine blood tests showed leukopenia and mild anemia. The pathologic result of mediastinal lymph node biopsy was granulomatous lesions; thus, the patient was diagnosed with type II sarcoidosis without glucocorticoid therapy. In the following 2 years, the patient suffered from intermittent fever accompanied by dyspnea, fatigue, occasional cough, less sputum, and apparent weight loss. Abnormal physical examinations included leg ulcers and splenomegaly. Laboratory and physical tests revealed pancytopenia, polyserositis, and enlargement of lymph nodes. The pathological findings of leg ulceration, pleura, and left supraclavicular lymph node all suggested granulomas.
Diagnosis, Interventions, and Outcomes
It strongly suggested sarcoidosis since tuberculosis, lymphoma, and connective tissue disease were all excluded. Due to severe conditions and multiorgan involvement, we tried to provide methylprednisolone for this patient. After 9 months of oral glucocorticoids therapy, his subjective symptoms as well as hematological and radiological findings were all improved. His leg skin ulceration and scab were also completely disappeared.
Conclusion
Sarcoidosis has diverse clinical presentations, and many patients present with atypical symptoms. It needs to be timely identified by the clinician and carefully differentiated from other diseases with similar findings so as to make an accurate diagnosis. In this case, the patient had a poor clinical response to glucocorticoids in the early stage of treatment due to the severe condition and multi-organ involvement. It is worth noting that the patient had improved significantly after 9 months of treatment of corticosteroids, which suggested that follow-up is critical.
