Chronic Lymphocytic Leukemia Presenting Research Articles

Racial Differences in the Presentation and Outcomes of Chronic Lymphocytic Leukemia and Variants in the United States

Chronic lymphocytic leukemia (CLL) is the most common form of adult leukemia in the United States, and prolymphocytic leukemia (PLL) is a related, rare chronic lymphoproliferative disorder. Using the United States Surveillance, Epidemiology and End Results (SEER) data from 13 registries, we examined differences in incidence and survival for CLL, small lymphocytic lymphoma (SLL) and PLL by race. International Classification of Diseases for Oncology 3(rd) edition histology codes 9670, 9823, and 9632-34 were used to identify cases. From 1992 to 2007, 30,622 cases of CLL/SLL and 268 cases of PLL were recorded. Males had higher incidence than females (male-to-female incidence rate ratio CLL/SLL 1.89, 95% confidence interval (CI) 1.85-1.94 and PLL 2.47, 95%CI 1.90-3.20). Black patients were diagnosed at younger age compared to white patients (mean age at diagnosis for white versus black patients for CLL/SLL, 70 versus 67 years, P < .001; for PLL, 71 versus 61 years, P < .001). Greater proportion of black patients with SLL presented with B symptoms, extranodal primary site, and advanced disease compared to white patients (P = .003, P = .012, and P = .009 respectively). White patients with CLL/SLL had better survival rates than black patients (5-year relative survival rate 77.1% versus 63.9%, P < .01). In univariate Cox regression models, black race, male gender, age at diagnosis > 65 years, advanced stage, and B-symptoms were predictors of worse survival (P < .01) among CLL/SLL patients. Black patients with CLL/SLL and PLL present at younger age and black patients with CLL/SLL have worse survival than white patients. Epidemiological studies examining the biological variants of these diseases in concert with race are needed to elucidate the etiology of these disparities.

Leukemia cutis in B-cell chronic lymphocytic leukemia presenting as an episodic papulovesicular eruption

A 53-year-old man presented with a recurrent pruritic eruption accompanied by oral sores. His past medical history was significant for subclinical B-cell chronic lymphocytic leukemia (CLL), which had never been treated. On exam, there were erythematous papules and plaques studded with vesicles on the neck, trunk, and upper extremities. Two skin biopsies showed common features of a perivascular and periadnexal lymphocytic infiltrate in the superficial to mid-dermis. Immunohistochemical staining of the lymphocytes showed co-expression of CD20, CD23, CD5, and CD43, consistent with a diagnosis of cutaneous involvement by the patient's CLL. This case highlights the importance of considering leukemia cutis in patients with underlying CLL presenting with unusual clinical features.

Subacute herpes simplex virus type 1 encephalitis as an initial presentation of chronic lymphocytic leukemia and multiple sclerosis: a case report

IntroductionHerpes simplex virus type 1 encephalitis presents acutely in patients who are immunocompetent. We report what we believe to be the first published case of a subacute course of herpes simplex virus type 1 encephalitis in a patient with asymptomatic chronic lymphocytic leukemia who subsequently developed multiple sclerosis.Case presentationA 49-year-old Caucasian woman with a history of fever blisters presented to the emergency department with a history of left temporal headache for four weeks, and numbness of the left face and leg for two weeks. A complete blood count revealed white blood cell count of 11,820 cells/mL, with an absolute lymphocyte count of 7304 cells/mL. The cerebrospinal fluid contained 6 white blood cells/μL, 63 red blood cells/μL, 54 mg glucose/dL, and 49 mg total protein/dL. Magnetic resonance imaging of the brain revealed meningoencephalitis and bilateral ventriculitis. Cerebrospinal fluid polymerase chain reaction for herpes simplex virus type 1 was positive, and the patient's symptoms resolved after ten days of treatment with parenteral aciclovir. Incidental findings on peripheral blood smear and flow cytometry testing confirmed chronic lymphocytic leukemia. One month later, she developed bilateral numbness of the hands and feet; a repeat cerebrospinal fluid polymerase chain reaction for herpes simplex virus type 1 at this time was negative. A repeat magnetic resonance imaging scan showed an expansion of the peri-ventricular lesions, and the cerebrospinal fluid contained elevated oligoclonal bands and myelin basic protein. A brain biopsy revealed gliosis consistent with multiple sclerosis, and the patient responded to treatment with high-dose parenteral steroids.ConclusionHerpes simplex virus type 1 encephalitis is a rare presentation of chronic lymphocytic leukemia. Our patient had an atypical, subacute course, presumably due to immunosuppression from chronic lymphocytic leukemia. This unusual case of herpes simplex virus type 1 encephalitis emphasizes the importance of T cell function in diseases of immune dysregulation and autoimmunity such as chronic lymphocytic leukemia and multiple sclerosis. It raises the question of whether atypical presentations of herpes simplex virus encephalitis warrant deliberations on immunocompetence. The development of multiple sclerosis in our patient so soon after she received treatment for herpes simplex virus type 1 encephalitis raises the possibility that herpes simplex virus type 1 encephalitis in an immunosuppressed patient may trigger multiple sclerosis.

Leukaemic infiltration of gall bladder - unusual presentation of occult chronic lymphocytic leukaemia.

Extramedullary involvement in early stage chronic lymphocytic leukaemia (CLL) is rare. We report the first case of an incidental finding of gall bladder infiltration in a patient who underwent a cholecystectomy for gallstone pancreatitis with no preceding history of CLL. This case reiterates the importance of subjecting even routine cholecystectomy specimens for histopathology examination in the context of this unusual presentation.

Flow cytometry for diagnosis of a rare case of chronic lymphocytic leukaemia presenting in the central nervous system and effective treatment with liposomal cytarabine

Flow cytometry for diagnosis of a rare case of chronic lymphocytic leukaemia presenting in the central nervous system and effective treatment with liposomal cytarabine

Cardiac tamponade mimicking tuberculous pericarditis as the initial presentation of chronic lymphocytic leukemia in a 58-year-old woman: a case report

IntroductionChronic lymphocytic leukemia is an indolent disease that often presents with complaints of lymphadenopathy or is detected as an incidental laboratory finding. It is rarely considered in the differential diagnosis of patients presenting with tamponade or a large, bloody pericardial effusion. In patients without known cancer, a large, bloody pericardial effusion raises the possibility of tuberculosis, particularly in patients from endemic areas. However, the signs, symptoms and laboratory findings of pericarditis related to chronic lymphocytic leukemia can mimic tuberculosis.Case PresentationWe report the case of a 58-year-old African American-Nigerian woman with a history of travel to Nigeria and a positive tuberculin skin test who presented with cardiac tamponade. She had a mild fever, lymphocytosis and a bloody pericardial effusion, but cultures and stains were negative for acid-fast bacteria. Assessment of blood by flow cytometry and pericardial biopsy by immunohistochemistry revealed CD5 (+) and CD20 (+) lymphocytes in both tissues, demonstrating this to be an unusual manifestation of early stage chronic lymphocytic leukemia.ConclusionAlthough most malignancies that involve the pericardium clinically manifest elsewhere before presenting with tamponade, this case illustrates the potential for early stage chronic lymphocytic leukemia to present as a large pericardial effusion with tamponade. Moreover, the presentation mimicked tuberculosis. This case also demonstrates that it is possible to treat chronic lymphocytic leukemia-related pericardial tamponade by removal of the fluid without chemotherapy.

Chronic Lymphocytic Leukemia Presenting with Cholecystitis-Like Symptoms and Gallbladder Wall Invasion

Although infiltration of the gallbladder by lymphoma is rare, it has to be considered in the differential diagnosis of patients presenting with cholecystitis-like symptoms. The most common lymphomas masquerading as acute cholecystitis are mucosa-associated lymphoid tissue lymphoma and large B-cell lymphoma. We describe a 75-year-old patient who presented with an acute cholecystitis-like picture, featuring chronic lymphocytic leukemia (CLL) with gallbladder wall involvement as the initial disease presentation. The cholecystectomy specimen showed perineural invasion present within the gallbladder wall, which likely accounted for the patient's right upper quadrant abdominal tenderness. In this way, we would like to alert clinicians and pathologists alike to CLL as yet another cause of a cholecystitis-like symptomatology.

Chronic Lymphocytic Leukemia Presenting With Localized Gynecologic Symptoms

Although no cure exists for chronic lymphocytic leukemia (CLL), treatment can reduce morbidity and/or improve survival in advanced disease. Identification of patients in an early disease state allows monitoring and treatment as soon as appropriate. We were unable to find previous reports of early CLL disease presenting with gynecologic symptoms. This report seeks to alert gynecologists of their potential role in identifying CLL patients. A 76-year-old woman presented with rectovaginal pressure. Cervical cone biopsy showed a lymphoproliferative process. Peripheral blood flow cytometry confirmed a diagnosis of early-stage CLL. The patient remains asymptomatic and is currently monitored for disease progression. Early CLL can present with localized gynecologic symptoms or cervical lesions. The recognition of early disease can assure optimal treatment.

Large B-cell transformation of chronic lymphocytic leukemia presenting as a penile mass and skin lesion

Hematol Oncol Stem Cell Ther 1(3) July 2008 hemoncstem.edmgr.com 199 A 77ayear old man was referred to our departa ment in January 2007 after developing a paina ful penile mass 2 months before. He had a history of chronic lymphocytic leukemia (CLL) for ten years. Two years before his referral he had received 12 courses of chlorambucil and prednisolone for the cona trol of resistant autoimmune hemolytic anemia. On physical examination there was splenomegaly (4 cm below the left costal margin) without palpable lymphadenopathy. Two violaceous nodular skin lesions with maximal diameters of 3 and 4 cm were evident in the left (Figure 1) and right forearm, respectively, as well as enlargement and hard infiltration of the base of the penis. Laboratory workaup revealed a positive dia rect antiaglobulin Coombs test (IgG) and the presence in the serum of IgGk monoclonal immunoglobulin. He had no anemia, thrombocytopenia or blood lymphocya tosis. A CT scan of the thorax and abdomen revealed a normal lung parenchyma and mediastinum, an absence of enlarged lymph nodes and only moderate splenoa megaly. The patient underwent sonographic examinaa tion of the penis. A large illadefined, heterogeneous, hypoechoic mass was shown infiltrating the corpora cavernosa. Color Doppler examination showed rich tumor vascularity. A pelvic MRI, using T2aweighted unenhanced and contrastaenhanced T1aweighted ima ages confirmed the presence of a mass infiltrating the corpora cavernosa and extending around the penile shaft with heterogeneous enhancement after contrast material administration (Figure 2). The mass invaded the corpus spongiusum, not extending to the urethra. Ultrasoundaguided penile biopsy and bilateral skin lesion biopsies were performed. Histopathologic exa amination revealed a diffuse infiltration by large Ba lympocytes (centroblast and immunoblast) (Figure 3). Tumor cells were CD45, CD20 and bcla6 positive and CD5, CD10, CD23, cyclin D3 and MUM1 negaa tive. A diagnosis of diffuse large Bacell lymphoma was Large B-cell transformation of chronic lymphocytic leukemia presenting as a penile mass and skin lesion

Clinical and immunological features in patients with chronic lymphocytic leukaemia presenting in an area of high incidence.

Summary. Newly presenting cases of chronic lymphocytic leukaemia in a retirement area were examined. Throughout a 27-month period these patients have been under the regular scrutiny of a small team who used standardized clinical and laboratory techniques for assessment. A definitive diagnosis and staging of the disease was made at the patient's first visit. The high incidence of the disease in this area is described and the influence of complications and coexistent disease on prognosis predicted by well-known staging technique is emphasized. Immunological techniques proved very useful in initial diagnosis, but their value in prognosis remains uncertain.

Bilateral Infiltrative Disease of the Extraocular Muscles: A Rare Clinical Presentation of Early Stage Chronic Lymphocytic Leukemia

Orbital involvement in chronic lymphocytic leukemia (CLL) is highly unusual and most commonly involves hemorrhage or soft tissue infiltration in advanced disease. We report a case of rapid onset bilateral orbital muscle infiltration as the presenting feature of early stage CLL. In addition, we demonstrate clinico-pathological correlation with an identical chronic B-cell lymphocytic infiltrate in both orbit and bone marrow, with good response of the orbital disease to local radiotherapy.

Hemorrhagic Pleural Effusion as a First Presentation of Chronic Lymphocytic Leukemia (A Case Report)

Hemorrhagic Pleural Effusion as a First Presentation of Chronic Lymphocytic Leukemia (A Case Report)

Massive scrotal oedema - an unusual primary presentation of Chronic Lymphocytic Leukaemia

Massive scrotal oedema - an unusual primary presentation of Chronic Lymphocytic Leukaemia

Successful treatment of a patient with chronic lymphocytic leukaemia (CLL) presenting with bony metastases with aggressive antibody and chemotherapy

Osteolytic lesions are rare in chronic lymphocytic leukaemia (CLL) and thought to result from Richter's transformation or metastatic disease from nonlymphoid malignancies. We report a patient who presented with a large femoral metastatic lesion and hypercalcaemia caused by CLL itself. Complete remission of CLL with resolution of the osteolytic lesion was achieved with rituximab and cyclophosphamide, adriamycin, oncovin and prednisolone [CHOP (R-CHOP)] combination chemotherapy.

Early B-cell chronic lymphocytic leukemia presenting as cutaneous lesions with a normal peripheral blood lymphocyte count

To the Editor: B-cell chronic lymphocytic leukemia (B-CLL) is similar to small lymphocytic lymphoma (SLL), having identical histopathologic and immunophenotypic features. However, a diagnosis of CLL requires a clonal population of typical morphology and immunophenotype in blood and bone marrow, even with normal lymphocyte counts.1 We report two cases of CLL presenting with only cutaneous infiltrates without lymphocytosis or lymphadenopathy.

Allergic reaction to chlorambucil in chronic lymphocytic leukemia presenting with fever and lymphadenopathy

Chronic lymphocytic leukemia (CLL) is commonly treated with the alkylating agent chlorambucil. Allergic reactions to this chemotherapy are rare. Previous reports include urticaria, angioedema, rashes, toxic epidermal necrolysis, drug fever and one case of immune hemolytic anemia. We report 2 cases that had the identical symptoms of acute onset of high fever and progressive lymphadenopathy. These symptoms disappeared with conservative management. Neither of the patients were treated for infection or disease progression. When the patients were rechallenged with chlorambucil, identical reactions recurred. There was no cross-reactivity with the alkylating agent cyclophosphamide or other types of chemotherapy. This type of reaction to chlorambucil has not been described previously. It is important to recognize this as an allergic reaction. These reactions could be confused with the onset of infection, progression of disease or even a Richter's transformation.

Alemtuzumab: effective monotherapy for simultaneous B‐cell chronic lymphocytic leukaemia and Sézary syndrome

The simultaneous presentation of chronic lymphocytic leukaemia (CLL) and cutaneous T-cell lymphoma (CTCL) is a very rare occurrence where optimal treatment is unknown. We present the case of a 65-yr-old man who was successfully treated with alemtuzumab monotherapy for both disorders, but at a cost of severe infectious morbidity and prolonged pancytopenia.

Nephrotic Syndrome Associated with Chronic Lymphocytic Leukaemia

The simultaneous presentation of B-cell chronic lymphocytic leukaemia and nephrotic syndrome is described. The leukaemia responded to cytotoxic therapy but prednisolone was required to achieve remission of the nephrotic syndrome. The nephrotic syndrome relapsed when the leukaemia again became active and the white cell count rose above 20 × 10 9 /l, and it remitted again with treatment. The relationship between the two diseases is discussed.

Serous retinal detachment in a case with chronic lymphocytic leukemia: no response to systemic and local treatment

Leukemias are systemic hematopoietic neoplasias and not infrequently cause ocular findings. Serous retinal detachment (SRD) is one of these manifestations and even may be the first sign of the underlying leukemia. Here we reported a case with chronic lymphocytic leukemia (CLL) presenting with SRD and discussed the clinical importance and therapeutic options.

Chronic Lymphocytic Leukemia Presenting with Extreme Hyperleukocytosis and Thrombosis of the Common Femoral Vein

Very few case reports dealing with chronic lymphocytic leukemia (CLL) and hyperleukocytosis have been reported in the medical literature and none with venous thrombosis as a complication. Here, we describe a 73-year-old woman who presented with newly diagnosed CLL, leukostasis, and hyperleukocytosis (2000 × 10 9 /l), affecting the respiratory and nervous system. In addition, she also had deep vein thrombosis (DVT). Although hypercoagulability and thrombosis are well-described phenomena in solid tumors and in myeloproliferative neoplasms, CLL is generally not associated with an acquired coagulopathy. We hypothesize that in our patient the extreme number of circulating lymphocytes resulted in an abnormal accumulation of lymphocytes possibly causing stasis and occlusion of a larger vessel, which resolved after leukopheresis. The patient has since been successfully maintained with chemotherapy. We conclude that leukopheresis should be considered as the therapy of choice in CLL patients presenting with major complications of leukostasis.