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Cureus Apr 2024Cryoglobulinemia may result in small-to-medium vessel vasculitis. Central nervous system (CNS) involvement is rare, and presentation may range from stroke/transient...
Cryoglobulinemia may result in small-to-medium vessel vasculitis. Central nervous system (CNS) involvement is rare, and presentation may range from stroke/transient ischemic attack, reversible ischemic neurological deficits, to encephalopathic syndromes. This is a rare case discussing cryoglobulinemia with CNS involvement. A 56-year-old female with a history of cryoglobulinemia was found unresponsive to verbal and physical stimuli. She was admitted to the intensive care unit. CT head without contrast showed diffuse cerebral edema and mass effect in the right cerebral hemisphere causing right to left midline shift, brainstem infarct, hemorrhage in the right lateral ventricle, and obstruction of the fourth ventricle. The patient was managed with hypertonic saline, external ventricular drain (EVD) placement, and high-dose steroids, which led to an improvement in her condition. In conclusion, testing for cryoglobulins and serologic tests for hepatitis C should be considered in syndromes of cerebral ischemia or infarction without an obvious cause, especially in young individuals since encephalopathy may be reversible. Cryoglobulinemia with CNS manifestations may be associated with purpura, high RF, and low C4. The treatment can be a combination of steroids, immunosuppressants, plasmapheresis, and rituximab. Cyclophosphamide may also be considered as adjunctive therapy to corticosteroids in rapidly progressive severe neurological complications. Further research for treatment standards in nonviral cryoglobulinemia is needed.
PubMed: 38752092
DOI: 10.7759/cureus.58259 -
Clinical and Experimental Vaccine... Apr 2024The coronavirus disease 2019 (COVID-19) vaccine was developed to provide immunity against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which was first...
The coronavirus disease 2019 (COVID-19) vaccine was developed to provide immunity against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which was first reported in 2019. The vaccine has proven to be effective in reducing severity and mortality and preventing infection. Henoch-Schönlein purpura is an autoimmune vasculitis (immunoglobulin A vasculitis). Historically, vaccines have been administered primarily to children, and Henoch-Schönlein purpura has often been reported in children following vaccination. However, since the start of COVID-19 vaccination, an increasing number of cases have been reported in adults. Here, we report a case of a patient who developed hematuria and proteinuria after receiving the messenger RNA COVID-19 vaccine. A 22-year-old man presented to the hospital with a lower extremity rash, bilateral ankle pain, and abdominal pain 18 days after receiving the COVID-19 vaccine. The man had no significant medical history and was not taking any medications. Laboratory tests showed normal platelet counts but elevated white blood cell counts and C-reactive protein and fibrinogen levels. He was treated with the non-steroidal anti-inflammatory drugs, pheniramine and prednisolone. At 40 days after starting treatment, C-reactive protein levels were within normal limits, and no hematuria was observed. Treatment was terminated when the purpura disappeared. This report is intended to highlight the need for further research to be proactive and carefully monitor for conditions associated with the COVID-19 vaccine.
PubMed: 38752010
DOI: 10.7774/cevr.2024.13.2.166 -
Surgical Case Reports May 2024Cases of abdominal aortic aneurysm discovered as purpura on the extremities with disseminated intravascular coagulation (DIC) are rare. The number of currently...
A case of abdominal aortic aneurysm presenting as symptomatic disseminated intravascular coagulation treated with endovascular aneurysm repair and postoperative administration of Nafamostat mesylate.
BACKGROUND
Cases of abdominal aortic aneurysm discovered as purpura on the extremities with disseminated intravascular coagulation (DIC) are rare. The number of currently established strategies for the control of DIC with aortic aneurysm is limited.
CASE PRESENTATION
An 85-year-old woman was referred to the hematology department because of purpura on her shoulder and thigh. Enhanced fibrinolytic-type DIC was diagnosed by a blood test. Enhanced computed tomography (CT) revealed 60-mm abdominal aortic and 42-mm right common iliac aneurysms. We performed endovascular aneurysm repair (EVAR) and coiling of the right internal iliac artery with postoperative administration of Nafamostat mesylate. The patient promptly recovered from DIC, and the purpura gradually disappeared.
CONCLUSIONS
We safely performed EVAR with postoperative administration of Nafamostat mesylate for an abdominal aortic aneurysms that presented as symptomatic DIC.
PubMed: 38750283
DOI: 10.1186/s40792-024-01926-6 -
BMC Nephrology May 2024Atypical haemolytic uremic syndrome (aHUS) is an uncommon form of thrombotic microangiopathy (TMA). However, it remains difficult to diagnose the disease early, given...
BACKGROUND
Atypical haemolytic uremic syndrome (aHUS) is an uncommon form of thrombotic microangiopathy (TMA). However, it remains difficult to diagnose the disease early, given its non-specific and overlapping presentation to other conditions such as thrombotic thrombocytopenic purpura and typical HUS. It is also important to identify the underlying causes and to distinguish between primary (due to a genetic abnormality leading to a dysregulated alternative complement pathway) and secondary (often attributed by severe infection or inflammation) forms of the disease, as there is now effective treatment such as monoclonal antibodies against C5 for primary aHUS. However, primary aHUS with severe inflammation are often mistaken as a secondary HUS. We presented an unusual case of adult-onset Still's disease (AOSD) with macrophage activation syndrome (MAS), which is in fact associated with anti-complement factor H (anti-CFH) antibodies related aHUS. Although the aHUS may be triggered by the severe inflammation from the AOSD, the presence of anti-CFH antibodies suggests an underlying genetic defect in the alternative complement pathway, predisposing to primary aHUS. One should note that anti-CFH antibodies associated aHUS may not always associate with genetic predisposition to complement dysregulation and can be an autoimmune form of aHUS, highlighting the importance of genetic testing.
CASE PRESENTATION
A 42 years old man was admitted with suspected adult-onset Still's disease. Intravenous methylprednisolone was started but patient was complicated with acute encephalopathy and low platelet. ADAMTS13 test returned to be normal and concurrent aHUS was eventually suspected, 26 days after the initial thrombocytopenia was presented. Plasma exchange was started and patient eventually had 2 doses of eculizumab after funding was approved. Concurrent tocilizumab was also used to treat the adult-onset Still's disease with MAS. The patient was eventually stabilised and long-term tocilizumab maintenance treatment was planned instead of eculizumab following haematology review. Although the aHUS may be a secondary event to MAS according to haematology opinion and the genetic test came back negative for the five major aHUS gene, high titre of anti-CFH antibodies was detected (1242 AU/ml).
CONCLUSION
Our case highlighted the importance of prompt anti-CFH antibodies test and genetic testing for aHUS in patients with severe AOSD and features of TMA. Our case also emphasized testing for structural variants within the CFH and CFH-related proteins gene region, as part of the routine genetic analysis in patients with anti-CFH antibodies associated aHUS to improve diagnostic approaches.
Topics: Humans; Still's Disease, Adult-Onset; Atypical Hemolytic Uremic Syndrome; Complement Factor H; Adult; Male; Autoantibodies; Macrophage Activation Syndrome
PubMed: 38745129
DOI: 10.1186/s12882-024-03548-4 -
Platelets Dec 2024Immune thrombocytopenia (ITP) is a common autoimmune hematological disorder. Despite this, diagnosis is still challenging due to clinical heterogeneity and the lack of a...
Immune thrombocytopenia (ITP) is a common autoimmune hematological disorder. Despite this, diagnosis is still challenging due to clinical heterogeneity and the lack of a specific diagnostic test. New findings in the pathology and the availability of new drugs have led to the development of different guidelines worldwide. In the present study, the Delphi methodology has been used to get a consensus on the management of adult patients with ITP in Spain and to help in decision-making. The Delphi questionnaire has been designed by a scientific ad hoc committee and has been divided into 13 topics, with a total of 127 items, covering the maximum possible scenarios for the management of ITP. As a result of the study, a total consensus of 81% has been reached. It is concluded that this Delphi consensus provides practical recommendations on topics related to diagnosis and management of ITP patients to help doctors to improve outcomes. Some aspects remain unclear, without consensus among the experts. Thus, more advances are needed to optimize ITP management.
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Spain; Delphi Technique; Consensus; Surveys and Questionnaires
PubMed: 38742687
DOI: 10.1080/09537104.2024.2336104 -
Rheumatology International Jul 2024IgA-associated vasculitis (IgAV) known as Henoch - Schönlein purpura (HSP) disease is an inflammatory disorder of small blood vessels. It's the most common type of... (Review)
Review
IgA-associated vasculitis (IgAV) known as Henoch - Schönlein purpura (HSP) disease is an inflammatory disorder of small blood vessels. It's the most common type of systemic vasculitis in children which can be associated with the inflammatory process following infections. IgA vasculitis is a rare and poorly understood systemic vasculitis in adults. Coronavirus disease 2019 (COVID-19) has been associated with HSP in both adults and children. A 58-year-old woman was diagnosed with HSP, fulfilling the clinical criteria: palpable purpura, arthritis, hematuria. The disclosure of the HSP disease was preceded by a infection of the respiratory tract. COVID-19 infection was confirmed via the presence of IgM and IgG antibodies. This case indicates the possible role of SARS-CoV-2 in the development of HSP. The clinical course of IgAV in adults appears to be different from pediatric IgAV, especially due to higher risk of renal complications. Symptoms of the disease quickly resolved with low-dose of steroids.
Topics: Humans; COVID-19; IgA Vasculitis; Female; Middle Aged; SARS-CoV-2; Immunoglobulin A
PubMed: 38739223
DOI: 10.1007/s00296-024-05606-4 -
Tidsskrift For Den Norske Laegeforening... May 2024
Topics: Humans; Purpura; Golf
PubMed: 38738575
DOI: 10.4045/tidsskr.24.0262 -
Cureus Apr 2024Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disorder characterized by hemolytic anemia, thrombocytopenia, renal...
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disorder characterized by hemolytic anemia, thrombocytopenia, renal failure, fever, and neurologic dysfunction. While cases often do not present with all five characteristics (<5%), TTP can be hereditary or acquired, often due to a deficiency or dysfunction of the ADAMST13 enzyme. Here, we describe a case of infection-induced acquired TTP in a middle-aged male with urinary tract infection (UTI) and perianal abscess. Suspicion arose from hematologic abnormalities, fever, thrombocytopenia, acute renal failure, and the presence of an underlying infection. A PLASMIC score of 6 (indicating a 72% probability of ADAMTS13 deficiency) prompted ADAMTS13 level testing, revealing levels <5% with the presence of an inhibitor, confirming TTP diagnosis. Treatment with high-dose steroids and daily plasma exchange yielded a swift platelet response, necessitating only two to three days of plasma exchange. In addition, incision and drainage of the perianal abscess were performed. The patient was discharged on daily prednisone and initiated on four doses of weekly Rituximab to mitigate recurrence risk. This case underscores the importance of early suspicion and treatment in infectious triggers such as UTI/perianal abscess, offering crucial diagnostic and prognostic insights.
PubMed: 38738139
DOI: 10.7759/cureus.57950 -
Cureus Apr 2024Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder typically presenting with a classic pentad of symptoms: thrombocytopenia, microangiopathic...
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder typically presenting with a classic pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction, and fever. This report explores an unusual presentation of TTP in a 47-year-old female with a medical history of hypertension, hyperlipidemia, and chronic TTP, who exhibited only petechial rashes, generalized weakness, and headache. Notably, the petechial rash, a less common manifestation of TTP, became a pivotal clue for the diagnosis, underscoring the necessity for vigilance even when classic symptoms are absent. This case reinforces the imperative of a high suspicion index for TTP, especially in patients with thrombocytopenia and hemolytic anemia, irrespective of other traditional signs. Plasmapheresis remains the treatment cornerstone, removing autoantibodies and replenishing ADAMTS13, as evidenced by the patient's initial response. The administration of rituximab, targeting B cells to mitigate autoantibody production against ADAMTS13, featured prominently in her management, aligning with its recognized role in refractory or relapsing TTP cases. Despite an encouraging response to rituximab, a subsequent decline in platelet count indicated the unpredictable nature of TTP and the necessity for multi-pronged therapeutic strategies. The patient's medical background and persistently low ADAMTS13 levels hinted at a chronic relapsing trajectory associated with increased morbidity and mortality. This necessitates ongoing vigilance and treatment flexibility. Highlighting this atypical TTP presentation, the report calls for immediate, robust intervention, serving as a critical reminder of the heterogeneity of TTP manifestations and the complexities in its management, thereby contributing to broader clinical awareness and improved patient prognoses.
PubMed: 38738084
DOI: 10.7759/cureus.57994 -
Cureus Apr 2024A 79-year-old man with type II diabetes mellitus and recently diagnosed idiopathic thrombocytopenic purpura presented to the Emergency Department with progressive...
A 79-year-old man with type II diabetes mellitus and recently diagnosed idiopathic thrombocytopenic purpura presented to the Emergency Department with progressive dyspnea over the course of two weeks. He was found to have diffuse miliary nodules, dense cavitary consolidation, and widespread cystic changes on chest imaging and died within 48 hours of admission to the hospital. His serum Coccidioides antibody and urine Histoplasma antigen were both positive. He later grew from the blood, supporting the theory that Histoplasma positivity was likely the result of antigen test cross-reactivity. Coccidioidomycosis typically presents with mild, self-limited symptoms, but may also disseminate rapidly, causing fulminant, life-threatening disease. Prompt recognition of risk factors for fulminant coccidioidomycosis and understanding flaws in serologic testing are essential to the appropriate diagnosis and management of this disease.
PubMed: 38738009
DOI: 10.7759/cureus.58129