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Tripathi, Preeti
- Amyloidosis Presenting as Severe Bleeding Diathesis
Abstract Views :216 |
PDF Views:127
Authors
Affiliations
1 Department of Hematology, All India Institute of Medical Sciences, New Delhi, IN
2 Dept of Pathology, Army Hospital Research and Referral, New Delhi, IN
1 Department of Hematology, All India Institute of Medical Sciences, New Delhi, IN
2 Dept of Pathology, Army Hospital Research and Referral, New Delhi, IN
Source
International Journal of Medical and Dental Sciences, Vol 6, No 2 (2017), Pagination: 1535-1538Abstract
Bleeding is one of the rare presentations of Amyloidosis. The mechanism behind spontaneous or peri- interventional bleeding in patients of amyloidosis is complex and involves multiple co-existing factors like coagulation factor deficiency, abnormal synthesis of coagulation factors due to advanced liver dysfunction, acquired Von Willebrand disease, platelet dysfunction, amyloid angiopathy and other unknown mechanisms. We present a case of middle aged female, presenting with spontaneous retroperitoneal haemorrhage, on further investigations was found to have systemic amyloidosis and secondary severe factor X deficiency (2.7 % of normal by one stage factor assay method). Factor X deficiency (both inherited and acquired) is known to present with the most severe bleeding phenotype. The management option for such acute spontaneous haemorrhage is limited and mostly supportive in nature. Definitive treatment is directed towards the primary pathology and requires chemotherapy and hematopoietic stem cell transplantation.Keywords
Amyloidosis, Bleeding, Factor X Deficiency, Platelet Dysfunction.Full Text
References
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- Bernard Soulier Syndrome; A Rare Bleeding Disorder
Abstract Views :223 |
PDF Views:136
Authors
Affiliations
1 All India Institute of Medical Sciences, New Delhi, IN
2 Department of Hematopathology, All India Institute of Medical Sciences, New Delhi, IN
1 All India Institute of Medical Sciences, New Delhi, IN
2 Department of Hematopathology, All India Institute of Medical Sciences, New Delhi, IN
Source
International Journal of Medical and Dental Sciences, Vol 7, No 1 (2018), Pagination: 1642-1645Abstract
Bleeding syndromes in the newborn are rare, but they may be life-threatening and demand immediate attention. Congenital bleeding disorders especially pose a diagnostic challenge to the clinician because of their rarity and the need to be differentiated from the other common causes of bleeding in children. We present a case of an infant presenting with bleeding symptoms early in his life (since 5 months of age) which was initially thought to be immune thrombocytopenic purpura (ITP) with low platelet count. No response to steroids and further evaluation by platelet aggregometry and flowcytometry led to the correct diagnosis – Bernard soulier syndrome(BSS). Though, there is no specific treatment available for this rare bleeding disorder, however it is imperative to have arrived at correct diagnosis in order to save unnecessary therapy and to take due precautions for prevention of bleeding.Keywords
Bernard Soulier Syndrome, Bleeding, Giant Platelets, Platelet Aggregation, Flowcytometry.Full Text
References
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- A Peripheral Smear Finding Beyond Cells: Cryoglobulins in a Case of Lymphoplasmacytic Lymphoma
Abstract Views :323 |
PDF Views:144
Authors
Affiliations
1 Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, IN
1 Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, IN
Source
International Journal of Medical and Dental Sciences, Vol 8, No 2 (2019), Pagination: 1744-1748Abstract
A well prepared peripheral smear is an indispensable requisite for the diagnosis of hematological disorders and in some situations, non-hematological diseases. Here, we present the typical hemogram and peripheral smear findings of circulating cryoglobulins that led to the unraveling of the underlying lymphoproliferative disorder. A 51-year lady presented with symptoms of anemia since four months. Her hemogram showed anemia and leucopenia with flagging of platelet count. Peripheral smear at room temperature revealed marked artefactual changes in red blood cells including fragmentation. The morphology was better appreciated after incubating the sample at 37°C and showed normocytic normochromic RBCs, thrombocytopenia with a few lymphoplasmacytic cells and amorphous pinkish material in the background. Bone marrow examination showed near total replacement of marrow spaces by lymphoid cells positive for CD20 and CD138 and was diagnosed as lymphoplasmacytic lymphoma. Cryoglobulins can thus cause a range of laboratory artefacts which need to be recognized, warranting further search for possible underlying etiologies.
Keywords
Artifacts, Cryoglobulinemia, Peripheral-Smear, Pseudothrombocytosis.Full Text
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