Kasabach Merritt syndrome in a 28-year-old female: A strange case report

Kasabach Merritt syndrome (KMS) is a rare disease in which a benign vascular tumor that is hemangioma grows rapidly, entraps red blood cells, platelets, and coagulation factors leading to activation of coagulation cascade resulting in life-threatening disseminated intravascular coagulation and microangiopathic hemolytic anemia. KMS affects newborns and infants. Rarely can affect older children and adults with only a few cases reported in the existing literature. Clinically patients present with large cutaneous hemangioma usually involving the extremities however visceral organs may be involved in some cases along with anemia, thrombocytopenia, coagulopathy, and bleeding. We report a case of KMS in a 28-year-old female who presented with bilateral subdural hematoma, thrombocytopenia, and consumption coagulopathy. She was given seven days course of methylprednisolone to which she responded well.

Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review

Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One rarely reported kidney lesion associated with benign paraproteins is thrombotic microangiopathy (TMA), provisionally considered as a combination signifying MGRS. Such cases may lack systemic features of TMA, such as a microangiopathic hemolytic anemia, and the disease may be kidney limited. There is no direct deposition of the paraprotein in the kidney, and the presumed mechanism is disordered complement regulation. We report three cases of kidney limited TMA associated with benign paraproteins that had no other detectable cause for the TMA, representing cases of MGRS. Two of the cases are receiving clone directed therapy, and none are receiving eculizumab. We discuss in detail the pathophysiological basis for this possible association. Our approach to therapy involves first ruling out other causes of TMA as well as an underlying B-cell malignancy that would necessitate direct treatment. Otherwise, clone directed therapy should be considered. If refractory to such therapy or the disease is severe and multisystemic, C5 inhibition (eculizumab or ravulizumab) may be indicated as well.

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy with clinical quintuple symptoms, including fever, thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, and renal insufficiency. TTP onset in children is rare, and the percentage of acute TTP with these five symptoms at the same time is <10%. In this study, we reported a typical case of TTP onset in a child with clinical quintuple symptoms.

Hemolytic Uremic Syndrome Caused by Mycoplasma Pneumoniae Infection in Children: A Case Report and Literature Review

We describe the case of a 6-year-old boy with a Mycoplasma pneumoniae (M. pneumoniae) respiratory tract infection associated with thrombotic microangiopathic hemolytic anemia and thrombocytopenia with renal failure which was diagnosed as atypical hemolytic uremic syndrome. Renal biopsy showed features of thrombotic microangiopathy. The patient was treated with azithromycin for the M. pneumoniae infection, and supportive care with red cell transfusion and renal dialysis in the acute period. The microangiopathic hemolytic anemia and thrombocytopenia resolved within 2 months after diagnosis but the renal function damage was irreversible. The patient developed end-stage renal disease and required long term renal replacement therapy.

Colon Cancer: An Unusual Presentation with a Paraneoplastic Syndrome

Dermatomyositis (DM) is a rheumatological disorder characterized by proximal myositis and distinctive dermatological manifestations. It can be an isolated clinical syndrome or, in rarer cases, can be the initial presentation for an underlying malignancy as a part of a paraneoplastic syndrome. In this case report, we describe a case of a 51-year-old lady who presented with proximal myopathy, typical DM skin rash, dysphagia, and markedly elevated creatine kinase. She was diagnosed with a seronegative DM and her malignancy screening revealed a mass in the ascending colon. During her hospital course, she also developed microangiopathic hemolytic anemia, another paraneoplastic disorder typically associated with late stages of malignancy, manifested as hemolytic anemia, thrombocytopenia, and low fibrinogen. The patient received intravenous corticosteroids and underwent tumor resection with following resolution of her both rheumatological and hematological manifestation. Unfortunately, due to her general poor health, she developed sepsis and died in the hospital.

A Rare Case of Thrombotic Thrombocytopenic Purpura Caused by Acute Pancreatitis

Thrombotic Thrombocytopenic Purpura is a syndrome of microangiopathic hemolytic anemia accompanied by thrombocytopenia, neurological disorders, renal failure and fever. Acute pancreatitis is a rare cause of Thrombotic Thrombocytopenic Purpura and this manifestation, at the same time, is a rare complication of acute pancreatitis. Thrombotic Thrombocytopenic Purpura is induced in acute pancreatitis by poorly understood mechanism, which involves multiple pathways apart from only ADAMTS-13 deficiency. Here, we analyze the case of a 47-year-old female who presented with an acute pancreatitis. She was diagnosed with Thrombotic Thrombocytopenic Purpura and an acute pancreatitis at the same time, with thrombocytopenia and peripheral smear findings at presentation. Therefore, Thrombotic Thrombocytopenic Purpura secondary to the pancreatitis was considered in this case. In this work, we have discussed details of our case and the different mechanisms involved in pathogenesis of Thrombotic Thrombocytopenic Purpura in acute pancreatitis and their outcome with prompt management.