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A patient with previously undiagnosed asymptomatic chronic lymphocytic leukaemia presenting with acute on chronic retention is reported. The relevant literature is discussed.
The authors, on the basis of a review of the literature and on their personal experience, derived from the observation of 169 cases of acute and chronic leukaemias, describe the different aspects of the cutaneous lesions present during the course of these neoplastic conditions. The cutaneous lesions, subdivided according to the type of leukaemia, show an extremely variable pattern and it is therefore difficult to differentiate on the basis of the sole macroscopic appearances, specific leukaemic nodules or infiltrates from dermatoses due to bacterial, viral and fungal infections on which an haemorrhagic component may be superimposed. Similarly, if a clearly established diagnosis of leukaemia has not been confirmed, it is difficult to distinguish leukaemic infiltrates from cutaneous lesions caused by a malignant lymphoma, metastatic carcinoma, an eosinophilic granuloma, or from benign lymphohistiocytic infiltrates, secondary to cutaneous infections. It is important also to bear in mind the development of skin eruptions, often with an haemorrhagic component, due to acquired sensitivity to numerous agents, cytostatic drugs, antibiotics, blood products and others, used in therapy. The authors therefore underline the importance of an early recognition of an infectious lesion, often complicated by a previous haemorrhagic event or secondary to microbial invasion of a catheter tip or of a venipuncture site, and consequently, the authors emphasize the need to distinguish these non-specific skin alterations, amenable to appropriate treatment from those lesions due to the development of a skin tumour or to a leukaemic infiltrate, which is nearly always indicative of a leukaemia recurrence or extension of the leukaemic process.
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We present a hybrid cellular automata-partial differential equation model of moderate complexity to describe the interactions between a growing tumor next to a nutrient source and the immune system of the host organism. The model allows both temporal and two-dimensional spatial evolution of the system under investigation and is comprised of biological cell metabolism rules derived from both the experimental and mathematical modeling literature. We present numerical simulations that display behaviors which are qualitatively similar to those exhibited in tumor-immune system interaction experiments. These include spherical tumor growth, stable and unstable oscillatory tumor growth, satellitosis and tumor infiltration by immune cells. Finally, the relationship between these different growth regimes and key system parameters is discussed.
A 72 year old man was diagnosed with chronic myelomonocytic leukaemia (CMML) according to the FAB group classification. He presented with symptoms of anaemia, urinary frequency, hesitancy, and nocturia. He was later admitted with acute urinary retention and acute renal failure, which resolved with treatment. A transurethral resection of the prostate was performed. Histological examination showed fibromuscular hyperplasia with dense infiltration by myelomonocytes which stained positively with chloroacetate esterase; immunohistochemical staining was positive for lysozyme, CD43, CD45, and CD68. Following treatment with oral etoposide he transformed to acute myeloid leukaemia and eventually died. Myelomonocytic infiltration of the prostate has not been reported before. This case extends the spectrum of disease previously recognised in CMML.
The testis is a common site of relapse in childhood acute lymphoblastic leukaemia (ALL). In adults, testicular relapses of ALL are very rare. A similar age-difference in the frequency of the testicular infiltration exists also in the rat T-cell leukaemia. In the present investigation, the effect of various hormonal treatments and unilateral cryptorchidism on the form of testicular infiltrates by the rat T-cell leukaemia was studied. Inhibition of testicular activity by estradiol treatment (E2) of early pubertal rats injected i.p. with rat T-leukaemic lymphoblasts significantly decreased the proportion of the testis occupied by leukaemic infiltrates. The proportion of the testis occupied by leukaemic infiltrates was significantly higher in the abdominal testes of both early and late pubertal unilaterally cryptorchid rats, than in the scrotal testes of leukaemic control rats. Daily treatment of early pubertal rats with human chorionic gonadotrophin (hCG), or human menopausal gonadotrophin (hMG), did not have an effect on testicular leukaemic infiltration. These studies demonstrate that the leukaemic infiltration of the testis is influenced by the changes in the physiological activity of the testes.
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A 36-year-old man had a high titer of antibody to Epstein-Barr virus (EBV) and recurrent necrotizing papules and nodules on his face and oral mucosa. The disease was diagnosed as CD3+4- 8+ large granular lymphocyte leukemia of T-cell origin. Southern blot analysis demonstrated that EBV DNA was present in CD8+ lymphocytes; EBV antigens were also observed in these lymphocytes. These findings demonstrated that EBV latently infected the leukemic cells and may have played a role in the pathogenesis of this disease. This is the first report of an association between EBV and large granular lymphocyte leukemia of T-cell origin.
Chronic lymphocytic leukemia (CLL) is a low-grade B-cell non-Hodgkin lymphoma. Skin involvement is observed in up to 25% of patients. A 70-year-old men with presented with prominent erythematous facial infiltrates. After the diagnosis of CLL was established, he showed a good hematologic response to the monoclonal antibody rituximab, but the skin infiltrates were therapy-resistant.
Ophthalmologists are familiar with the commonly observed leukemic retinopathy and orbital infiltration in patients with both acute and chronic leukemia. With the advent of more successful and aggressive chemotherapeutic regimens and more sophisticated radiation therapy, the eye is not uncommonly involved in patients who are in either meningeal or hematological relapse. In rare instances, ophthalmologic involvement may be the only manifestation of recurrence of the leukemic process. In addition, retinal microaneurysms, capillary closure, and neovascularization have been documented in individuals with chronic leukemia. This paper will review leukemic retinopathy and leukemic infiltration of the orbit. In addition, a discussion of the frequency and pathogenesis of the retinal vascular abnormalities will be presented. The clinical picture and treatment of optic nerve, choroid and iris infiltration will be discussed. Unusual ocular manifestations as the presenting sign of leukemia will also be detailed.
Juvenile chronic myelogenous leukaemia (JCML) is a rare haematological malignancy of myelomonocytic lineage that affects patients less than 4 years of age and is known as an entity different from adult-type chronic myelogenous leukaemia. In JCML, skin manifestations are relatively common but most of them have been reported as a non-specific eruption, which histologically may show changes resembling neurofibromatosis or xanthogranuloma. We present a 2-year-old boy with JCML who developed a recurrent annular erythema in which leukaemic infiltrates were confirmed histologically, even though his bone marrow examination suggested that be remained in haematological remission.
An unusual presentation of acute lymphoblastic leukaemia (ALL) in a 6-year-old girl is reported. She presented with unilateral cervical lymphadenopathy, a mixed obstructive/cholestatic jaundice and a progressive pancytopenia. Ultrasound examination revealed an obstructed common bile duct with gross thickening of the wall of the duct and intrahepatic bile duct dilatation. The jaundice resolved with high dose intravenous (i.v.) methylprednisolone. It is postulated that this was due to infiltration of the common bile duct, given the failure to demonstrate any other cause for the bile duct pathology.
We describe 2 elderly patients with splenomegaly and progressive bone marrow failure due to infiltration by leukemic prolymphocytes with a single prominent nucleolus. In each case the leukemia cells had a unique helper T cell phenotype with complement receptors and contained coarse blocks of acid alpha-naphthyl butyrate. A 14q+ chromosome was among the abnormalities that marked one abnormal T cell clone. Histology of the spleen showed mainly red pulp infiltration merging with periarteriolar regions and different from the pseudonodular pattern described in many cases of B cell prolymphocytic leukemia (PLL). Ultrastructurally, leukemia cells contained dense lysosomes, perinuclear clusters of 10-nm microfilaments but no distinctive cytoplasmic inclusions previously described in (B cell) PLL. We suggest that early phenotyping of leukemia cells may enable consideration of monoclonal antibody therapy in elderly patients with bone marrow failure resistant to chemotherapy.
Congenital leukemia and leukemoid reactions may be indistinguishable on clinical and histologic grounds and are highly associated with trisomy 21. This report characterizes a specific vesiculopustular skin eruption in an infant with Down syndrome and a congenital leukemoid reaction. On the first day of life an unusual vesiculopustular eruption developed, starting in areas of cutaneous trauma. A biopsy revealed immature myeloid cells in an epidermal spongiotic vesiculopustule and in a perivascular distribution, suggestive of leukemia cutis. As the peripheral blood smear normalized, the eruption cleared. Myelodysplasia subsequently developed and evolved into acute myelogenous leukemia. This is the first detailed report of a specific skin infiltrate caused by the immature cells of a leukemoid reaction. Skin infiltration by immature myeloid cells during a congenital leukemoid reaction may portend an aggressive course of the myeloproliferative disorder.
Cutaneous presentation of B-cell chronic lymphocytic leukaemia (B-CLL) is uncommon, and the influence of skin changes on B-CLL prognosis is unclear. We report a patient with B-CLL Rai II, with multiple nodular skin infiltrations on the trunk, upper arms and thighs as well as constitutional symptoms, who was successfully treated with cladribine. The peripheral blood (PB) lymphocytes were CD19, CD20, CD23 and CD5 positive, which confirmed the diagnosis of B-CLL. Skin biopsy of one of the lesions showed an intense infiltrate composed of small lymphocytes with no epidermotropism. These cells also showed the expression of CD19, CD20, CD23 and CD5 antigens similar to those presented on PB lymphocytes. Polymerase chain reaction performed on bone marrow lymphocytes and a lesional skin biopsy using consensus primers for immunoglobulin heavy-chain genes also showed the same monoclonal population of B lymphocytes both in the bone marrow and in the skin. The patient received four courses of cladribine 0.12 mg kg-1 daily as a 2-h infusion for five consecutive days. The courses were repeated at monthly intervals. The lymphocytosis gradually decreased and the PB count normalized after three courses. At the same time, a significant decrease in the cutaneous symptoms was observed. The patient became free of skin tumours after the fourth course of cladribine; only slight discoloration at the previous sites of cutaneous infiltration remained. There was no relapse of leukaemia cutis during a further 7 months of observation.
BACKGROUND: Specific cutaneous infiltrates in patients with leukemia generally carry a grim prognosis. However, non-neoplastic skin diseases may be associated with recruitment of normal and neoplastic leukocytes circulating in the peripheral blood. In those instances, neoplastic cells may be detected in skin lesions without an adverse effect on prognosis. METHODS: In a patient with B-cell chronic lymphocytic leukemia, a specific infiltrate developed at the site of a florid herpes simplex infection. Clinically, the lesion presented itself as an ulcerated tumor. RESULTS: Histopathologically, the lesion was characterized by a dense, diffuse infiltrate of small hyperchromatic lymphocytes throughout the entire dermis. Lymphocytes showed an aberrant CD20(+)/CD43(+)/CD5(+) phenotype of neoplastic B cells, and monoclonal rearrangement of immunoglobulin gamma genes could be demonstrated by polymerase chain reaction. Although criteria for leukemia cutis were fulfilled, the patient did well. CONCLUSIONS: The cutaneous infiltrate of neoplastic cells seemed to be part of a physiologic response to the antigenic stimulus, rather than indicating an exacerbation of leukemia. Ziemer M, Bornkessel A, Hahnfeld S, Weyers W. 'Specific' cutaneous infiltrate of B-cell chronic lymphocytic leukemia at the site of a florid herpes simplex infection.
A 3-year-old Japanese domestic cat with a diagnosis of lymphocytic leukemia showed severe generalized seizures in the course of chemotherapy after leukemic condition was improved clinically. Computed tomography (CT) and magnetic resonance (MR) imaging of the brain were carried out. Both contrast procedures disclosed enhancements at the falx cerebri and the margin of cerebral cortex. Among these procedures contrast MR imaging demonstrated the lesion most clearly. Cytological examination of cerebrospinal fluid obtained by spinal puncture showed the infiltration of malignant cells and the diagnosis of meningeal syndrome associated with lymphocytic leukemia was defined. Intrathecal administration of cytosine arabinoside partially improved the neurologic dysfunction. Autopsy and histopathological examination confirmed the infiltration of leukemic cells in the areas of meningeal lesion demonstrated with contrast CT and MR imaging. Thus these imaging techniques, especially contrast MR imaging, are useful tools for rapid and precise diagnosis of meningeal syndrome.
The case report presents a 35-year-old patient with neurological disorders caused by a solid tumour of the spinal cord and organ infiltration during the chronic phase of CML (chronic myelogenous leukemia). In spite of combined chemo- and radiotherapy, disease progression was only halted for a short time. Patient death occurred 5 months after disease diagnosis. Taking into consideration the non-typical disease course and few described case reports available in literature concerning solid tumours located in the CNS (central nervous system) with organ infiltration it appears noteworthy to discuss thoroughly the above case report.