scholarly journals Hydroa Vacciniforme-Like Lymphoproliferative Disorder in an Adult Patient With Chronic Lymphocytic Leukemia

2020 ◽  
Vol 11 (11) ◽  
pp. 366-369
Author(s):  
Ashlyn Chee ◽  
Benjamin Wood ◽  
Genevieve Sadler ◽  
Gavin Cull
Blood ◽  
1990 ◽  
Vol 76 (11) ◽  
pp. 2360-2367
Author(s):  
CA Hanson ◽  
TE Gribbin ◽  
B Schnitzer ◽  
JA Schlegelmilch ◽  
BS Mitchell ◽  
...  

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.


Blood ◽  
1990 ◽  
Vol 76 (11) ◽  
pp. 2360-2367 ◽  
Author(s):  
CA Hanson ◽  
TE Gribbin ◽  
B Schnitzer ◽  
JA Schlegelmilch ◽  
BS Mitchell ◽  
...  

Abstract Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Smitha Narayana Gowda ◽  
Hafez Mohammad Abdullah ◽  
Rakshya Sharma ◽  
Mohamed A. Abdallah

Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder in the United States. It has a variable presentation with most patients having asymptomatic lymphocytosis. Many other patients present with lymphadenopathy or enlargement of other organs of the reticuloendothelial system. However, CLL can present with extramedullary involvement. Most commonly, this is in the form of skin or central nervous system involvement, though rarely it can present with gastrointestinal involvement. Here, we present the case of a 70-year-old Caucasian male who presented with chronic diarrhea for over 4 months. After failing conservative treatment, a colonoscopy was performed which showed diffuse mucosal nodularities with a biopsy revealing CLL. The patient was treated successfully with chemotherapy and his diarrhea improved. This is a differential to keep in mind in patients with chronic diarrhea, once the more common causes have been ruled out.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 961-961
Author(s):  
Miguel Marin ◽  
Libia Sanz ◽  
Elena Sarsotti ◽  
Isana Benet ◽  
Maria J. Terol ◽  
...  

Abstract Introduction: Chronic lymphocytic leukemia B (CLL-B) is the most frequent chronic lymphoproliferative disorder in western countries. The majority of patients are diagnosed in incipient Binet stage A. Some prognostic factors have been identified, as mutations in the genes coding for immunoglobulin variable regions (IgVH). Complementary somatic mutations in the BCL6 gene have been observed in 25% of CLL-B patients, although its clinical relevance remains unclear. Objective: To identify molecular markers of different patient groups of lymphoproliferative disorder through analysis of their proteomic profiles. Material and Methods: 15 samples of peripheral blood lymphocytes B from Binet stage A CLL-B patients were analyzed. Among them, 5 carried mutations in IgVH and BCL6 (M) genes, 5 had no mutations (IgVH, BCL6 (U)), and 5 with mutations in IgVH(M) but without mutations in BCL6(U). 100 μg of total proteins were isoelectrofocussed using DryStrips (13 cm, pI 4–7), followed by 15% SDS-PAGE, and Coomasie Blue staining. Image acquisition and analysis were performed with the Image Master Platinum software (Amersham Biosciences). Spots showing differential expression were subjected to automatic tryptic digestion. Proteins were identified by MALDI-TOF mass fingerprinting using the Protein Prospector software. Selected peptide ions were sequenced by collision-induced dissociation (CID) using an ESI-QTRAP instrument. Results: The mean number of spots per gel was 214, which showed a mean percentage matching among the three patient groups of 77%. Statistics of the 2D-IEF/SDS-PAGE separations of the total lymphocyte B protein from patients with different mutational status of their IgVH and BCL6 genes. IgVH, BCL6 (M) vs. IgVH(M), BCL6(U) IgVH (M), BCL6 (U) vs. IgVH, BCL6(U) IgVH, BCL6 (M) vs. IgVH, BCL6(U) (*) Test t of student. Spot media per gel 228/180 180/235 228/235 Matching percentage 80.2% 79.3% 70.5% Number of spots with p<0.05(*) 6 2 2 MALDI-TOF mass fingerprint and/or CID MS/MS identification of spots with differential expresion among the 3 patient groups, and their level of expression in each mutational situation (Center value of volume % in each of the gels). Protein IgVH, BCL6(M) IgVH(M), BCL6(U) IgVH, BCL6(U) ns: no statistical significance 14-3-3 Protein Z (*) 0.82 0.30 ns Heterochromatin-specific nonhistone protein (*) 0.29 0.02 ns NADH-Ubiquinone reductase (*) 0.12 0.02 ns Tubulin α (*) 0.54 1.28 ns Vimentin (*) 0.27 0.98 ns Similar Beta Tubulin (*) 0.40 1.85 ns Cytoskeleton associated protein 1 (**) ns 0.03 0.26 Endoplasmatic-reticulum-lumenal protein 28 (**) ns 0.55 0.21 Chaperonin TCP-1 0.005 0.05 0.18 Nucleophosmin 0.17 1.51 1.22 Conclusions: Proteomic profiling of lymphocyte proteins of the three patient groups revealed 10 proteins with differential expression, which can be divided into the following categories: The expression of six proteins (*), changed with the mutational status of BCL6 gene. The expression of two proteins (**), correlated with the mutational status of the IgVH gene. Nucleophosmin exhibited increased expression in patients with unmutated BCL6 gene. Chaperonin TCP-1 expression was higher in patients with unmutated IgVH gene. Work supported by Redes Tematicas de Grupo Grant G03/179 of the Instituto de Salud Carlos III, Madrid, Spain


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1770-1770
Author(s):  
Edouard Cornet ◽  
Alice-Sophie Boucher ◽  
Véronique Salaun ◽  
Florence Truquet ◽  
Michele Malet ◽  
...  

Abstract Flow cytometry is the diagnostic tool of choice to study abnormal lymphoid population detected in peripheral blood by morphological analysis. The main diagnosed chronic lymphoproliferative disorder (CLPD) is chronic lymphocytic leukemia (CLL). In a significant number of cases, a B-CLPD non-CLL can be diagnosed. Further molecular and histological examinations are then compulsory to characterize such hematologic malignancies. The objective of this study was to determine the incidence of atypical CLL among all B-CLPD diagnosed by flow cytometry. We retrospectively studied the B-CLPD consecutively diagnosed at the hospital of Caen (Normandy, France) between 2000 and 2013. The diagnosis of B-CLPD was based on the detection by flow cytometry of circulating lymphoid abnormal B cells. Multiparametric flow cytometry included markers CD19, CD20, CD22, CD79b, CD5, CD10, CD23, CD43, FMC7, CD38 and light chains (kappa and lambda) of surface immunoglobulin. The diagnosis of CLL was based on the criteria defined by Hallek et al (Blood 2008). The non-CLL B-CLPD were then explored by molecular analyses driven by the phenotype of B-cells (overexpression of cyclin D1 in case of CD5+/CD10-/CD23- B-CLPD and BCL2-JH rearrangement in case of CD10+ B-CLPD). In addition, histological evidence was necessary to classify the B-CLPD non-CLL. 1819 B-CLPD were detected by flow cytometry. The distribution of B-CLPD was as follows: 1156 cases (64%) of CLL or immunophenotypic equivalent (leukemic phase of small lymphocytic lymphoma (SLL) and monoclonal B-cell lymphocytosis (MBL)), 297 cases (16%) of marginal zone lymphoma (MZL), 84 cases (5%) of mantle cell lymphoma (MCL), 39 cases (2%) of follicular lymphoma (FL), 26 cases (1%) of hairy cell leukemia (HCL), 13 cases (<1%) of diffuse large B-cell lymphoma (DLBCL), 9 cases (<1%) of Waldenstrom's macroglobulinemia (WM) and 3 cases (<1%) of B-cells prolymphocytic leukemia (B-PLL). 65 cases (4%) remained unclassified due to lack of histological and molecular data. 127 cases (7%) did not meet the diagnostic criteria of CLL established by Hallek et al but were classified as atypical CLL because of the detection of a clonal B-cell proliferation expressing CD5+ / CD23+ / CD43+ / CD10- / FMC7+ / CD79b+ with moderate or high intensity and light chain kappa or lambda with moderate or strong intensity (absence of molecular or histological argument of MZL or MCL was required). CD20 marker was highly expressed in 113 cases (89%) of atypical CLL. We particularly studied the 1532 cases (84%) of B-CLPD expressing CD5 (table). CD5+ CD23+ B-CLPD cases accounted for 1293 (84%) with 1153 cases of CLL, 13 cases of MCL and 127 cases of atypical CLL. CD5+ CD23- B-CLPD accounted for 239 cases (16%) with 72 cases of MCL, 158 cases of MZL, 4 cases of FL, 2 cases of WM, 2 cases of CD23- CLL and one case of B-PLL.CD5+ casesCD5+ CD23+ B-CLPDCD5+ CD23- B-CLPDTotalCLL115301153Atypical CLL1272129MCL137285MZL0158158FL044WM022B-PLL011Total12932391532 WHO classification of hematologic malignancies do not include atypical CLL as defined by a clonal proliferation of B-cells expressing CD5+, CD23+, cyclin D1- with no histological evidence of MZL or MCL, and which do not meet all the diagnostic criteria of CLL (Hallek et al, Blood 2008). This concept of atypical CLL, first described by Criel et al (BJH 1997), is particularly interesting, because such B-CLPD seems to have a different outcome as compared with CLL (Oscier et al, BJH 1997) and to have a different biological presentation with atypical morphology of CLL cells (Criel et al, BJH 1997), more frequent trisomy 12 (Matutes et al, BJH 1996) and a stronger intensity of CD20 (Ugo et al, Leuk Lymphoma 2006). Diagnosis of B-CLPD relies on a multidisciplinary approach combining morphological, immunophenotypic, molecular and histological analyses. Despite detailed information of these analyses, there are B-CLPD which remain unclassifiable according WHO classification, especially CD5 positive B-CLPD. The concept of atypical CLL seems to take all its meaning to help define such unclassifiable entities. Disclosures: No relevant conflicts of interest to declare.


2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Diego Velasco-Rodríguez ◽  
Miguel Piris-Villaespesa ◽  
Carmen Soteras ◽  
Ana Vallés ◽  
José Antonio García-Marco ◽  
...  

Chronic lymphocytic leukemia (CLL) is an incurable lymphoproliferative disorder with a heterogeneous genetic and clinical course. Two kinase inhibitors, ibrutinib and idelalisib, have demonstrated achievement of complete and durable remissions in relapse/refractory genetically unselected CLL patients. We present a case of relapsed CLL with extensive disease and hourglass deformity of urinary bladder as a result of the compression of two extraperitoneal paravesical soft tissue bulky masses, with excellent response to sequential kinase inhibitor therapy.


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