Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
The etiology is unknown. Factors such as circulating toxins, immune deregulation, or abnormalities of prostacyclin metabolism with low serum levels have been incriminated (prostacyclin is normally secreted by the vascular endothelium and has an anti-aggregating effect on thrombocytes). TTP can be related to various infections: viral, Mycoplasma pneumoniae, Bartonella-like infection, vaccination, subacute bacterial endocarditis. The partially occluded vessels damage the erythrocytes and thrombocytes which pass through them. The clinical signs will be those related to the microangiopathic hemolytic anemia, the thrombocytopenic purpura, and the obliterated vessels in the particular organs. The fever, which is present in 90% of patients, may be due to lesions in the hypothalamus.
Thrombotic Microangiopathy
Hau C. Kwaan, Meyer M. Samama in Clinical Thrombosis, 2019
Three major events occur in this condition: hemolysis, thrombocytopenia, and altered functions of various visceral organs. Their occurrence may be explained on the basis of the microthrombotic lesions. The partial occlusion of the arterioles and capillaries allows the passage of blood through altered hemodynamic conditions. Red blood cells flowing through the involved vessels with fibrin covering the thrombi are traumatized and fragmented, forming schistocytes and resulting in hemolysis (Figure 2). This alteration was first demonstrated by Brain et al.34 who termed the process microangiopathic hemolytic anemia. The thrombocytopenia follows excessive platelet consumption due to aggregation and platelet thrombi formation.
Therapeutic apheresis
Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond in Transfusion Medicine in Practice, 2020
The presentation of TTP is classically described by a clinical pentad of (i) microangiopathic hemolytic anemia (MAHA), (ii) thrombocytopenia; (iii) renal failure, (iv) fever, and (v) mental status changes. In practice, the presence of thrombocytopenia (<100 × 109/l) and an elevated lactate dehydrogenase (LDH > 1000 units/l, a manifestation of systemic ischemia and hemolysis) in the absence of another likely cause is sufficient to make the diagnosis.45,46 Although this disease was once associated with a mortality rate in excess of 80%, following the introduction of daily plasma exchange the mortality rate has been reduced to 10–20%.
Cobalamin and folic acid deficiencies presenting with features of a thrombotic microangiopathy: a case series
Published in Acta Clinica Belgica, 2022
Britt Ceuleers, Sofie Stappers, Jan Lemmens, Lynn Rutsaert
‘Microangiopathic hemolytic anemia’ is a term used to describe any Coombs-negative hemolytic anemia resulting from red cell fragmentation in the small vessels, identified as schistocytes and fragmentocytes on peripheral blood smears. When these abnormalities are accompanied by thrombocytopenia, one should be aware of thrombotic microangiopathies. This group of related disorders, including thrombotic thrombocytopenia and hemolytic-uremic syndrome, is characterized by thrombosis in the microvasculature due to an endothelial injury, resulting in the aforementioned nonimmune hemolytic anemia and associated end-organ damage [1–3]. Here, we introduce three cases of vitamin B12 and/or folic acid deficiencies, presenting with features of a thrombotic microangiopathy. In this manner, we aim to make the reader aware of this notorious but rare manifestation of these nutritional deficiencies, hopefully leading to an earlier recognition of these pseudo-thrombotic microangiopathies (pseudo-TMA). Hence, unnecessary and expensive treatment, such as plasmapheresis, should be avoided or at least shortened in duration.
Beneficial effect of fresh frozen plasma in reducing renal complications in Hemiscorpius lepturus scorpion envenomated children with severe hemoglobinuria: an open label randomized clinical trial
Published in Toxin Reviews, 2023
Ehsan Valavi, Parisa Amoori, Neda Mohtasham, Tahereh Ziaei Kajbaf, Mehri Taheri, Bahman Cheraghian, Soodeh Hooshmandi
In our previous study, acquired ADAMT13 deficiency was highly prevalent in patients with significant hemoglobinuria due to HL envenomation and was effective in the development of HUS (Valavi et al. 2011, 2014). ADAMTS13 deficiency is one of the major causes of atypical HUS. Without ADAMTS13, von Willebrand factor (VWF) multimers can grow to pathologically high molecular weights. This contributes to the unlimited growth of microvascular thrombi, leading to clinical manifestations of microangiopathic hemolytic anemia (Tersteeg et al. 2015). However, it has been recently reported that ADAMTS13 activity decreases in conditions, such as snake bite, liver cirrhosis, idiopathic thrombocytopenic purpura, leukemia, chronic uremia, HUS, DIC, systemic lupus erythematosus, pregnancy, postoperative state, neonatal period, and advancing age (Levy et al. 2005, Isbister et al. 2007, Malbranque et al. 2008).
Splenic Hemangiomatosis: An Uncommon Vascular Lesion in an Infant
Published in Fetal and Pediatric Pathology, 2018
Gargi Kapatia, Suvradeep Mitra, Kirti Gupta, Prema Menon, K. L. N. Rao
Vascular tumors of spleen represent a wide spectrum of lesions, ranging from benign to highly aggressive neoplasms. Among the benign tumors, localized hemangiomas are the commonest. Splenic hemangiomatosis by definition is considered to be a diffuse variant of hemangioma, characterized by total or subtotal replacement of its parenchyma by a vascular proliferation featuring the usual morphological findings of hemangioma [1]. This condition may occur synchronously with hemangiomatosis of the liver [1] or it can be a part of generalized angiomatosis involving different viscera, skin, retroperitoneum, bone marrow, or lymph nodes [2]. Isolated diffuse hemangiomatosis of the spleen is an uncommon lesion described in the literature as case reports. Moreover, pediatric examples are infrequent with isolated case reports [3,4]. Hematologic abnormalities, including anemia, thrombocytopenia, and severe coagulopathies, such as microangiopathic hemolytic anemia are common accompaniments. We describe another example of this lesion in an infant boy who presented with abdominal distension and during the work-up was found to have splenic hemangiomatosis.
Related Knowledge Centers
- Blood Smear
- Hemolytic Anemia
- Microangiopathy
- Microscopy
- Schistocyte
- Thrombotic Thrombocytopenic Purpura
- Cancer
- Hemolytic–Uremic Syndrome
- Disseminated Intravascular Coagulation
- Lupus