+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report

, : Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report. Bmc Research Notes 7(): 495-495

Acquired angioedema is a rare but recognized manifestation of lymphoproliferative disorders due to deficiency in C1 esterase inhibitor. Normal level of C1 esterase inhibitor proteins in association with angioedema due to lymphoproliferative disease is a rare and an uncommon finding caused by antibodies produced from the underlying disease. Antibodies cause inactivation of C1 esterase inhibitor, thus resulting in C1 esterase inhibitor dysfunction despite of normal quantity of C1 esterase inhibitor. A 50-year-old Sri Lankan male presented with first episode of angioedema without any family history. Physical examination revealed mild pallor with swelling of tongue, lips and perioral region. On investigations, erythrocyte sedimentation rate was persistently high and bone marrow with immunohistochemistry revealed infiltration with B-cell type low grade non-Hodgkin lymphoma. Computed tomography scan of the chest and abdomen showed paratracheal and subcarinal lymphadenopathy and splenomegaly, with the findings being compatible with lymphoma. He had normal C1 esterase inhibitor protein level with reduced activity and low C1q, C4 levels indicating antibodies against C1 esterase inhibitor causing dysfunctional C1 esterase inhibitor. Adult onset angioedema should prompt physicians to suspect underlying lymphoproliferative disorder despite of C1 esterase inhibitor protein level being normal. Though uncommon, presence of antibodies against C1 esterase inhibitor secondary to lymphoproliferative disorder should be considered in the presence of normal C1 esterase inhibitor protein levels with low functional capacity in the background of acquired angioedema.


PMID: 25099363

DOI: 10.1186/1756-0500-7-495

Other references

Stäubli, H.U.; Jakob, R.P., 1983: Fractures in the knee joint region: errors, hazards, complications. Therapeutische Umschau. Revue Therapeutique 40(11): 973-977

Cruz, R.G.nskopp, D., 1998: Seasonal preferences of steers for prominent northern Great Basin grasses. The objective of this research was to determine, on a seasonal basis, the relative preferences of cattle for 7 native grasses and d crested wheatgrass (Agropyron desertorum (Fischer ex Link)Schultes), a long-used introduction in the Pacific Northw...

Elie Aoun; Abdul Baki, H.; Azar, C.; Fadi Mourad; Kassem Barada; Zeina Berro; Mohsen Tarchichi; Sharara, A.I., 2005: A randomized single-blind trial of split-dose PEG-electrolyte solution without dietary restriction compared with whole dose PEG-electrolyte solution with dietary restriction for colonoscopy preparation. Colonoscopy preparation regimens are poorly tolerated, requiring the use of a large volume of an unpalatable solution and diet restriction for adequate cleansing. The aim of this study was to compare the efficacy of two regimens of bowel preparati...

Connors P.G.; Myers J.P.; Connors C.S.W.; Pitelka F.A., 1981: Inter habitat movements by sanderlings calidris alba in relation to foraging profitability and the tidal cycle. Sanderlings (C. alba) wintering near Bodega Bay, California [USA] move between outer coast sandy beaches and nearby harbor sandflats on a regular tidal schedule. Birds forage on outer beaches at high and mid-level tides, switching to the protected...

Shaikh, A., 1978: Urinary incontinence during treatment with depot phenothiazines. British Medical Journal 1(6128): 1698-1698

Bosin T.R.; Lundman A.; Beck O., 1983: Identification and quantitation of 6 hydroxy 1 2 3 4 tetra hydro beta carboline in alcoholic beverages. Journal of Agricultural & Food Chemistry 31(2): 444-446

Anonymous, 1961: The National Library Of Medicine Anniversary Issue. Bulletin of the Medical Library Association 49(3): 466-468

Bohm, M.; Gonzalez Fernandez, M.Isabel.; Ozen, S.; Pistorio, A.; Dolezalova, P.; Brogan, P.; Barbano, G.; Sengler, C.; Klein-Gitelman, M.; Quartier, P.; Fasth, A.; Herlin, T.; Terreri, M.Teresa.R.A.; Nielsen, S.; van Rossum, M.A.J.; Avcin, T.; Castell, E.Rodolfo.; Foeldvari, I.; Foell, D.; Kondi, A.; Koné-Paut, I.; Kuester, R-Michael.; Michels, H.; Wulffraat, N.; Amer, H.Ben.; Malattia, C.; Martini, A.; Ruperto, N., 2014: Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis). Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paedi...

Lee, Y.H.n; Kim, H.S.ok; Pai, J.K.on, 1994: Activation of phospholipase D induced by platelet-derived growth factor is dependent upon the level of phospholipase C-g1. The Journal of Biological Chemistry 269: 842-7

Senn H.; Loosli H R.; Sanner M.; Braun W., 1990: Conformational studies of cyclic peptide structures in solution from proton nmr data by distance geometry calculation and restrained energy minimization. The three-dimensional structure of a cyclic bouvardin analogue, cyclo(-Pro-MeTyr-Ala-MeTyr-MeTyr-D-Ala-) has been determined by distance geometry calculations and restrained energy minimization from nmr data. The preparation of the input for the d...