scholarly journals Larynx Angioedema as a Signal in Chronic Lymphocytic Leukemia: A Case-based Guide for Acquired Angioedema in the Setting of Lymphoproliferative Disorders

2021 ◽  
Vol 19 (2) ◽  
pp. 110-113
Author(s):  
Zeynep Büşra Kısakürek ◽  
Sadi Can Sönmez ◽  
Emre Osmanbaşoğlu ◽  
Erman Öztürk ◽  
Ayşe Bilge Öztürk
Keyword(s):  
Emergency Medicine ◽  
Chronic Lymphocytic Leukemia ◽  
Complete Blood Count ◽  
Primary Diagnosis ◽  
Emergency Department Visits ◽  
Lymphocytic Leukemia ◽  
Acquired Angioedema ◽  
Allergy And Immunology ◽  
Life Threatening ◽  
One Year

ABSTRACT Patients with angioedema can present to the internal medicine, emergency medicine, dermatology, or ear nose throat clinics. Physicians may need to assess the patients whose angioedema is unresponsive to antihistamines systematically in collaboration with other subspecialties including hematology, rheumatology, allergy, and immunology. We aimed to provide a concise review of the diagnosis and multi-disciplinary management of acquired angioedema through a case presentation. A 61-year-old woman presented with recurrent angioedema of 4 episodes within one year. She was evaluated by various disciplines such as dermatology and emergency medicine. Antihistamines and steroids were not effective. The complete blood count (CBC) results indicated lymphocytosis (lymphocyte count=9100 k/μL) and further evaluation of the lymphocytosis with flow cytometry immunophenotyping confirmed a diagnosis of chronic lymphocytic leukemia. Since the acquired angioedema diagnosis was confirmed with low C4, C1q, and C1 esterase inhibitor levels, Rituximab 375 mg/m2 was administered once a week for 4 weeks. The frequency of attacks decreased after rituximab therapy and none of them were life-threatening. In conclusion, when the effective treatment is initiated for the primary diagnosis in acquired angioedema, the numerous emergency department visits, hospitalizations, and the mortality due to life-threatening angioedema episodes can be avoided. Keywords: Angioedema, larynx angioedema, emergency, chronic lymphocytic leukemia, life-threatening angioedema

scholarly journals Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Abdullateef Abdulkareem ◽  
Ryan S. D’Souza ◽  
Joshua Mundorff ◽  
Pragya Shrestha ◽  
Oluwaseun Shogbesan ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Chronic Lymphocytic Leukemia ◽  
Upper Airway ◽  
Recurrent Abdominal Pain ◽  
Final Diagnosis ◽  
Lymphocytic Leukemia ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL) who presented to our hospital with recurrent abdominal pain, initially suspected to haveClostridium difficilecolitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema.

Early Autologous Stem Cell Transplantation (SCT) in Genetically Poor-Risk Chronic Lymphocytic Leukemia Is Feasible and Effective: Results from a Prospective Multicenter Study (GCLLSG CLL3 Protocol).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 146-146 ◽  
Author(s):  
Peter Dreger ◽  
Raimonde Busch ◽  
Stephan Stilgenbauer ◽  
Hildegard Greinix ◽  
Manfred Hensel ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Single Case ◽  
Progression Free Survival ◽  
Multicenter Trial ◽  
Lymphocytic Leukemia ◽  
Hematological Toxicity ◽  
High Dose ◽  
Post Transplant ◽  
Poor Risk ◽  
One Year

Abstract From July 1997 through June 2002, the German CLL Study Group conducted a prospective multicenter trial to assess the feasibility and efficacy of early SCT in patients with poor-risk chronic lymphocytic leukemia (CLL). The protocol comprised optional cytoreduction with CHOP, fludarabine, or FC; PBSC mobilization using the Dexa-BEAM regimen; and myeloablative therapy with TBI/CY followed by reinfusion of purged stem cells. Inclusion criteria were age <61 years, stage Binet B/C or poor-risk stage A as defined by short lymphocyte doubling time plus elevated STK, and one line of pretreatment or less. Endpoints were feasibility (as defined by the proportion of patients able to successfully undergo all study procedures); toxicity; efficacy; and quality of life (QOL) as defined by the Spitzer QOL Index. Results: As of July 2004, 128 of 179 eligible patients were evaluable. Median age was 51 (27–60) years. Binet stages were poor-risk A 15%; B 61%; C 24%. An unmutated VH rearrangement was present in 72%. Eighty-six percent of the patients were chemotherapy naive at study entry. Best documented response during the pretransplant phase was complete remission (CR) in 28% and partial remission (PR) in 64% of the patients, giving an overall response rate of 92%. Immunomagnetically purged grafts were obtained in 83% of the patients in whom mobilization was attempted, containing 4.6 (1.2–22.1) CD34+ cells per kg body weight. Altogether, 98 of 128 patients (77%) proceeded to SCT. Reasons for exclusion from SCT were disease progression, mobilization failure, toxicity during the mobilization phase, or patient’s refusal. At 3 months post SCT CR was reported in 78% and PR in 21%. With a follow-up time of 36 (3–77) months, median progression-free survival of all 128 patients intended to treat was 59 months (54 months for those with unmutated VH). 4-year overall survival of all 128 patients was 84% (95%CI 74–94). Grade 3/4 non-hematological toxicity was mainly due to infections, which occurred in 21% of the patients (18% pretransplant; 7% post transplant). Six complications were fatal (4 pretransplant, 2 post transplant), translating into a 5% probability of treatment-related death. A single case of treatment-related myelodysplasia / AML has been reported to date. One year after SCT, QOL index had the maximum score of 10 points in 48 of 57 evaluable patients (84%). In comparison to prestudy levels, QOL had increased in 35%, remained similar in 50%, and decreased in 15% of the patients. Conclusions: Early sequential high-dose therapy including SCT for poor-risk CLL is feasible and has promising efficacy. Whereas the transplant-related toxicity appears to be acceptable, future studies should aim at replacing Dexa-BEAM by a similarly effective but less toxic mobilization regimen.

Global DNA Hypermethylation in Chronic Lymphocytic Leukemia Correlates with Progressive Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5006-5006
Author(s):  
Margaret K. Yu ◽  
Aniko Szabo ◽  
Hector Bergonia ◽  
Anna Senina ◽  
John D. Phillips
Keyword(s):  
Dna Methylation ◽  
Chronic Lymphocytic Leukemia ◽  
Progressive Disease ◽  
Lymphocytic Leukemia ◽  
Global Dna Methylation ◽  
Dna Hypomethylation ◽  
Physician Visits ◽  
Mutational Status ◽  
Trisomy 12 ◽  
One Year

Abstract Global DNA hypomethylation is observed in chronic lymphocytic leukemia, but methylation has not been correlated with clinical outcome. Although patient survival correlates with factors such as the mutational status of the immunoglobulin variable genes, karyotype abnormalities, Zap-70, and CD38 expression, none of these predictors have altered the way clinicians practice. We report interim results in the assessment of global DNA methylation as a predictor of aggressive disease in patients with chronic lymphocytic leukemia. Fourteen patients with chronic lymphocytic leukemia donated blood samples for DNA studies at the same time as blooddraws for their physician visits. All the treatments occurred within one year and the follow-ups were at least within 12 months except for one patient. We thus classified patients into two groups: those who required treatment within one year and those who did not. The cutoff in methylation level providing the smallest observed prediction error was 4.125%; it correctly predicted 5/6 patients not needing treatment within a year and 5/6 patients needing treatment. These observed classification rates were adjusted for the bias resulting from the optimal selection of the cutoff using bootstrap. The adjusted sensitivity and specificity were 74% and 80%, respectively. Asymptomatic patients with chronic lymphocytic leukemia tended to have lower levels of global DNA methylation (median 3.5%) compared to symptomatic patients (median 4.5%). In other words, high levels of global DNA methylation were associated with higher disease burden, corresponding with higher lymphocyte and white blood cell numbers. Five patients without immediate need for cytoreductive therapy were enrolled on a pilot treatment trial with low-dose cladribine, by subcutaneous injection. Three out of the five patients have had a clinical response, a secondary endpoint. Two of the patients have achieved a partial response, as defined by the NCI sponsored working group, with at least a three month follow-up after discontinuation of the drug. Of the two patients with stable or progressive disease on cladribine, their global DNA methylation levels were higher, correlating with more chemotherapy resistant disease. DNA methylation Levels in Patients with Chronic Lymphocytic Leukemia Age Sex %5-MedC Zap-70 CD-38 Rai Stage FISH Req Treatment (mos) FISH= fluorescence in situ hybridization; Zap-70 assessed by immunochemistry 51 M 5.045 positive positive 4 Del13q14 0.75 73 F 4.865 positive not assessed 4 not assessed 12+ 52 F 4.665 positive negative 2 Del13q14 2 57 M 4.62 negative negative 4 Del13q14 2 66 M 4.59 positive not assessed 4 Del13q14 (5/05) and Del 17p and Del 13q14 (7/05) 0.25 67 M 4.14 positive positive 4 Trisomy 12 10 47 M 4.055 negative negative 0 not assessed 12+ 59 M 3.9 negative negative 2 46XY 0 72 M 3.54 negative not assessed 0 not assessed 8+ 64 M 3.55 positive not assessed 1 Del13q14 12+ 70 F 3.47 not assessed negative 4 Trisomy 12 12+ 68 F 3.47 positive negative 2 not assessed 12+ 77 M 3.2 not assessed not assessed 0 not assessed 12+ DNA Methylation Levels in patients with Chronic Lymphocytic Leukemia DNA Methylation Levels in patients with Chronic Lymphocytic Leukemia

scholarly journals The Auto-Immune Hemolytic Anemia of Malignant Lymphocytic Disease

Blood ◽  
1955 ◽  
Vol 10 (3) ◽  
pp. 197-227 ◽  
Author(s):  
MARTIN C. ROSENTHAL ◽  
ANTHONY V. PISCIOTTA ◽  
ZACHARIAS D. KOMNINOS ◽  
HENRY GOLDENBERG ◽  
WILLIAM DAMESHEK
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Hemolytic Anemia ◽  
Primary Diagnosis ◽  
Lymph Node Biopsy ◽  
Lymphocytic Leukemia ◽  
Coombs Test ◽  
Laboratory Findings ◽  
Cell Counts ◽  
Immune Hemolytic Anemia ◽  
The Difference

Abstract Twenty-four cases of malignant lymphocytic disease associated with frank hemolytic anemia are reviewed. Four cases were associated with some form of lymphosarcoma and twenty cases appeared in conjunction with chronic lymphocytic leukemia. In the latter cases, the hemolytic process was detected in eleven patients from one-half to five years after the original diagnosis had been established. In three patients, a positive Coombs’ test antedated the development of any other evidence of a hemolytic process by either several months or a year. In a significant number of cases, x-ray therapy seemed to precipitate the appearance of the abnormal hemolysis. In nine other patients, only when symptoms referable to hemolytic anemia necessitated medical aid, was the lymphocytic leukemia detected. Of the four patients with lymphosarcoma, three presented themselves initially as cases of hemolytic anemia. The primary diagnosis was uncovered only after lymph node biopsy or laparotomy. The physical examination in both disease groups represented a combination of the findings associated with hemolytic anemia and lymphocytic disease. In the cases of chronic lymphocytic leukemia, this led to splenomegaly of unusual degree and disproportionate to the size of the lymphadenopathy. The laboratory findings likewise combined the features of both the primary lymphocytic disorder and hemolytic anemia. Anemia, reticulocytosis, spherocytosis, and bilirubinemia of the indirect type were present, and in the cases of lymphocytic leukemia, elevated white cell counts with diminished platelet counts were found. The bone marrow in many instances represented a "duality of proliferation", hyperplasia of erythroid and lymphoid elements existing side by side. Immunologic studies clearly indicated that the mechanism of hemolysis in these cases differed in no respect from that of "idiopathic" auto-immune hemolytic anemia. The direct Coombs’ test was positive in all twenty cases in which it was employed, and circulating hemagglutinins were demonstrated in eleven of nineteen cases so tested. The multiplicity of laboratory indications of hemolysis, as well as the distinct immunologic mechanism, distinguish these cases of hemolysis from the so-called "occult" hemolytic anemia. The term "overt" hemolytic anemia has been used to emphasize the difference. The role of the lymphoid apparatus in the production of such auto-immune hemolytic anemia is discussed. The bulk of evidence would seem to implicate some cellular component of the lymphoid system, although not the adult lymphocyte, as the producer of antibody. The architectural disruption of this system in malignant lymphocytic disease might well have a functional counterpart with respect to its immunologic duty. The resultant aberration may manifest itself clinically as auto-immune hemolytic anemia. At present the therapy of choice for control of the hemolytic process is ACTH, Compound E or Compound F. Transfusions and splenectomy are useful ancillary measures when hormonal therapy is either partially or totally unsuccessful. The hemolytic component should be treated independently of the lymphocytic proliferation since control of both cannot be established by any one agent currently available.

scholarly journals Pulmonary Mucormycosis in Chronic Lymphocytic Leukemia and Neutropenia

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Izza Mir ◽  
Sijan Basnet ◽  
David Ellsworth ◽  
Elan Mohanty
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Fungal Infection ◽  
Amphotericin B ◽  
Thoracoscopic Surgery ◽  
Left Lung ◽  
Lymphocytic Leukemia ◽  
Pulmonary Mucormycosis ◽  
Life Threatening ◽  
History Of ◽  
Video Assisted Thoracoscopic Surgery

Pulmonary mucormycosis is a rare life-threatening fungal infection associated with high mortality. We present the case of a 61-year-old man with history of chronic lymphocytic leukemia who presented with fever and cough, eventually diagnosed with pulmonary mucormycosis after right lung video-assisted thoracoscopic surgery. The patient was successfully treated with amphotericin B and right lung pneumonectomy; however, he later died from left lung pneumonia.

Refractory acquired angioedema in chronic lymphocytic leukemia

2020 ◽  
Vol 61 (13) ◽  
pp. 3280-3281
Author(s):  
Avani Singh ◽  
Vijeyaluxmy Motilal Nehru ◽  
David Peace
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Lymphocytic Leukemia ◽  
Acquired Angioedema

scholarly journals Chronic lymphocytic leukemia in general practice

2021 ◽  
Vol 27 (1-2) ◽  
pp. 40-46
Author(s):  
Ljiljana Đurović
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Blood Count ◽  
Complete Blood Count ◽  
Clinical Symptoms ◽  
Pathological Finding ◽  
Lymphocytic Leukemia ◽  
Medical Council ◽  
Time Space ◽  
Breath Sounds ◽  
Number Of Patients

Introduction: Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults, rarely affecting children. It is more common in males over 60. Etiopathogenetically, it represents an abnormal proliferation of lymphocytes in the bone marrow, which are dysfunctional although morphologically similar to mature ones. Case report: Female patient, 66, a housewife, comes for an examination due to fatigue lasting about a month and weight loss of 3-4 kg. She denies other health problems. On the examination, she is alert, oriented to time, space, and persons, eupnoeic, lymphadenopathy on the neck, axillae and groins. She gives away the impression of a patient with mild clinical symptoms. Clinical examination: clear breath sounds in all lung fields, regular heartbeat, clear tones, BP 140/80 mmHg, abdomen at chest level, painless to palpation, liver not palpable, and spleen palpated 2 cm below the costal arch. CBC (complete blood count) showed an increased number of leukocytes 181.30x109 /L and lymphocytosis 92.2%, other parameters were normal. She was referred to the hematologist in the Kraljevo General Hospital, where further diagnostics were performed. Blood count was repeated, Chest X-ray was performed, ultrasound (US) of the abdomen neck, axillae, groins, and heart, virology tests, ENT examination. Abdominal US showed a pathological finding with enlarged liver, spleen, lymph nodes (LN). ENT examination: enlarged tonsils, other findings unremarkable. Since chronic lymphocytic leukemia was suspected, she was referred to the Clinical Center of Serbia. Immunophenotyping (IF) and computed tomography (CT) of the neck, chest, and abdomen were performed. The diagnosis of CLL was confirmed. A Medical Council decided to perform immunochemotherapy (IHT) with fludarabine and rituximab. After 6 received cycles, the patient feels well and the CBC parameters are normal. Conclusion: CLL may be asymptomatic or nonspecific and with mild symptoms in a large number of patients. Therefore, blood tests with leukocytosis and absolute lymphocytosis findings are crucial to suspect the disease and perform further diagnostics.

scholarly journals Coexistence of trisomy 12 and del(13)(q14.3) in two patients with chronic lymphocytic leukemia

2009 ◽  
Vol 61 (4) ◽  
pp. 607-611
Author(s):  
Marija Dencic-Fekete ◽  
D. Antic ◽  
Sanja Davidovic-Mrsic ◽  
Ivana Franic ◽  
Nada Kraguljac-Kurtovic ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Prognostic Significance ◽  
Lymphocytic Leukemia ◽  
Stable Phase ◽  
Fish Analysis ◽  
Fluo Rescence ◽  
Trisomy 12 ◽  
One Year ◽  
The Given

We describe two patients with diagnosis of chronic lymphocytic leukemia (CLL) in whom interphase fluo?rescence in situ hybridization (FISH) analysis revealed trisomy 12 and del(13)(q14.3) occurring in the same clone. These abnormalities are rarely seen together and the prognostic relevance of their coexistence is still unclear. According to some data, a probable adverse prognosis for this group of patients is suggested. Our patients have been in a stable phase of the disease for more than one year since the given abnormalities were documented in their karyotypes. Further study is necessary to determine the prognostic significance of coexistence of these abnormalities in CLL patients.

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