Transfusion of the Patient with Congenital Coagulation Defects

Shusterman, Suzanne; Manno, Catherine S.

doi:10.1016/b978-0-443-06981-9.50039-9

Cited by 4 publications

(2 citation statements)

References 111 publications

(152 reference statements)

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“…Normal PT, INR, aPTT, and SF ruled out deficiencies in both the intrinsic pathway and extrinsic pathway (and common pathway) of the coagulation cascade. Excessive bleeding with no anatomic, hematological, and biochemical abnormalities along with typical presentation of bleeding after a particular time period (48 h in our case) 5 guided us to evaluate clot stability 6 . Clot solubility in 5 M Urea was negative after 24 h. Factor XIII (FXIII) is the only coagulation factor that does not get involved in the intrinsic and extrinsic pathway but only aids in clot stability.…”

Section: Diagnosis Treatment Outcome and Follow‐upmentioning

confidence: 79%

Post‐dental extraction bleeding: Emphasis on the diagnosis of rare coagulation disorders

Sharma

I³

et al. 2021

Clinical Case Reports

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Disorders of the fibrin stabilizing and fibrinolytic pathway should be considered in patients with excessive postsurgical bleeding with normal screening tests of hemostasis. History, clinical assessment of the timing and severity of bleeding along with utilization of advanced tests such as global hemostasis assays and appropriate coagulation factor assays (especially FXIII) will aid in the diagnosis.

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Section: Diagnosis Treatment Outcome and Follow‐upmentioning

confidence: 79%

Post‐dental extraction bleeding: Emphasis on the diagnosis of rare coagulation disorders

Sharma

I³

et al. 2021

Clinical Case Reports

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“…Factor X in the form of fresh frozen plasma and prothrombin complex concentrates (PCCs) can be administered as needed for severe bleeding and trauma, as well as before major surgical procedures. PCCs are useful when sustained hemostasis is necessary and would require large volumes of plasma infusions [ 15 ]. Clinicians should keep in mind the possible risk of developing PCC-induced thrombosis [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

2022

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Light chain (AL) amyloidosis is a lethal form of systemic amyloidosis that arises from the clonal expansion of CD38+ plasma cells. Organ damage occurs when these plasma cells produce misfolded immunoglobulin light chains, which form amyloid fibrils and deposit in tissues. A minority of patients with AL amyloidosis show “raccoon eyes” caused by increased vascular fragility from accumulation of amyloid fibrils. Amyloidosis can be directly associated with bleeding diathesis due to factor X deficiency as factor X binds to amyloid fibrils primarily in the liver and spleen. A 65-year-old Caucasian male presented with random bruising in the upper chest and around the eyes for 1.5 years. Physical examination was unremarkable, except for neck bruising. Pertinent workup showed protein electrophoresis with a faint M spike, increased serum lambda light chains, a kappa to lambda ratio of 0.06, increased Bence-Jones proteins, reduced factor X activity, elevated NT-proBNP. The bone marrow biopsy was positive for Congo red stain for amyloid protein. Magnetic resonance imaging revealed diffuse enhancement of the right and left ventricle subendocardial late gadolinium, consistent with cardiac amyloidosis. The patient started systemic therapy with a regimen of daratumumab, cyclophosphamide, bortezomib, and dexamethasone. After one cycle of therapy, lambda light chains normalized with an improvement in bruising. Diagnostic delays for cardiac patients are concerning as the median survival rate among these patients, when not treated, is approximately 6 months after the onset of symptoms. Since timely treatment can prevent organ damage, clinicians should be aware of specific clinical signs such as raccoon eyes and the importance of systemic evaluation for a prompt diagnosis.

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Blood Coagulation

Baldo¹

2016

Safety of Biologics Therapy

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Transfusion of the Patient with Congenital Coagulation Defects

Cited by 4 publications

References 111 publications

Post‐dental extraction bleeding: Emphasis on the diagnosis of rare coagulation disorders

Post‐dental extraction bleeding: Emphasis on the diagnosis of rare coagulation disorders

Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis

Blood Coagulation

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