Diffuse osteosclerosis in hairy cell leukemia

We describe two patients with a new clinical pathologic syndrome of diffuse osteosclerosis in association with hairy cell leukemia. In both patients bone marrow biopsies could not be obtained due to extremely hard bones and inability to insert the biopsy needle; neither patient had a history of bony pain or fracture. The osteosclerotic process in one patient stabilized after successful treatment of her hairy cell leukemia with interferon alpha and deoxycoformycin suggesting that the osteosclerosis observed was related to the underlying malignant disease. Possible etiologic mechanisms are discussed.     

Acute liver failure in a patient with hairy cell leukemia

Acute liver failure as a manifestation of primary non-Hodkin's lymphoma is a rare phenomenon with a fatal prognosis. Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder, representing about 2 percent of all leukemies. We report a 78-year-old patient with a history of hairy cell leukemia since 10 years, presenting whith fulminant liver failure due to massive liver infiltration. He have reviewed several cases of infiltration of the liver by haematological malignancies, but we only have found after a review in MEDLINE between 1980 and 2006, one case of acute liver failure in a patient with hepatic invasion by hairy cell leukaemia.     

毛细胞白血病及伴毛细胞的脾淋巴瘤

毛细胞白血病 (ｈａｉｒｙｃｅｌｌｌｅｕｋｅｍｉａ ,ＨＣＬ)最早被称为白血病网状内皮细胞增生症。于 195 8年由Ｂｏｕｒｏｎｃｌｅ等首先提出。根据毛细胞的表面免疫球蛋白确定ＨＣＬ属于成熟Ｂ淋巴细胞增殖性疾患 ,成熟阻滞阶段发生在慢性淋巴细胞白血病和多发性骨髓瘤之间。其特点是骨髓和外周血有多毛细胞并伴有全血细胞减少、明显的脾脏肿大 ,而淋巴结不肿大。ＨＣＬ是一少见的疾病 ,仅占白血病的 2 %。欧美发病率较高。有人报告美国男性发病率为每年 2 9/百万人口 ,女性每年 0 6 /百万人口 ,男女比例为 4∶1。中位发病年龄5 2岁。…     

Diffuse large B-cell non-Hodgkin's lymphoma in a patient with autoimmune lymphoproliferative syndrome

Mutations of Fas or Fas ligand genes result in the autoimmune lymphoproliferative syndrome (ALPS) in humans. We report here a diffuse large B-cell non-Hodgkin's lymphoma occurring in a man with ALPS. Fas-mediated lymphocyte apoptosis was defective in vitro, owing to a mutation within the death domain of the Fas molecule. High-dose methotrexate and doxorubicin-based chemotherapy led to complete remission of lymphoma.     

Development of hairy cell leukemia in a patient with antiphospholipid syndrome

We present a young woman who was diagnosed as primary antiphospholipid syndrome (deep vein thrombosis and pulmonary embolism in 1999; moderate thrombocytopenia with high-positive anticardiolipin ELISA tests in 2002, and cerebral thrombosis in 2003), and then developed hairy cell leukemia (massive splenomegaly, neutropenia, hairy cells in blood smear and bone marrow trephine biopsy in 2004). A partial remission was achieved with interferon-alpha 2a therapy. After the initiation of 2-chloro-deoxyadenosine therapy, splenomegaly disappeared, the percentage of hairy cells on the bone marrow reduced below 1%, platelet count returned to normal levels. After complete remission was achieved for hairy cell leukemia proved by bone marrow trephine biopsy, antiphospholipid antibodies were found to be negative, and no further thromboembolic complications and thrombocytopenia were seen. In our literature search, we found only six cases that had both antiphospholipid antibodies and hairy cell leukemia. Our case is the first case of antiphospholipid syndrome before the development of hairy cell leukemia. Both hairy cell leukemia and antiphospholipid syndrome responded to lymphocytotoxic treatment with 2-chloro-deoxyadenosine.     

Immunoglobulin gene rearrangements in hairy cell leukemia

Studies of hairy cell leukemia have yielded conflicting data about the cell of origin in this disease. To investigate this issue, we have examined the state of immunoglobulin genes in the cells of 11 randomly selected spleens showing histologic involvement with hairy cell leukemia. DNA was extracted from splenic tissue samples and digested with restriction endonucleases. Following agarose gel electrophoresis and transfer to nitrocellulose filters or activated nylon membranes, splenic DNA was hybridized with radiolabeled DNA fragment probes specific for the constant regions of the immunoglobulin heavy chain and kappa and lambda light chain genes. Autoradiograms of the hybridized DNA in each case revealed rearrangements of a heavy chain gene and at least one light chain gene. In addition, immunophenotyping of cellular immunoglobulin polypeptides was carried out on frozen tissue sections from all but one case. In each case in which an immunoglobulin polypeptide could be detected, a rearrangement was present in the DNA of the corresponding immunoglobulin gene. These studies offer strong evidence for endogenous immunoglobulin synthesis in hairy cells and for the B lymphocytic character of this leukemia.     

Diffuse large B cell lymphoma: molecular targeted therapy

Diffuse large B cell lymphoma (DLBCL) is a biologically heterogeneous disease and the most common subtype of B cell non-Hodgkin??s lymphoma in the USA. Even though it is a curable lymphoma in advanced stages, up to 40?% of patients eventually relapse or fail to achieve remission. Improved understanding of the biologic complexity of DLBCL reveals a diverse range of oncogenic driver mutations and signaling pathways that are essential for growth and survival of malignant cells. Since many of these signaling pathways can be targeted by small-molecule inhibitors, the therapy for DLBCL is currently undergoing a paradigm shift away from conventional chemotherapy and toward targeted agents that capitalize on an improved biologic understanding of the subsets with the highest risk of treatment failure. Participation in well-conducted and rationally designed clinical trials will be essential to realize the potential of these targeted agents and realize the goal of improving overall outcomes in the most common B cell lymphoma in the world.     

Community-acquired pneumonia caused by Legionella dumoffii in a patient with hairy cell leukemia

Summary A case of community-acquired pneumonia caused byLegionella dumoffii in a patient with hairy cell leukemia is described. Diagnosis was confirmed by isolation by culture of sputum and broncho-alveolar lavage specimens, positive direct fluorescent antibody stains, and antibody seroconversion from 1 : 16 (acute) to 1 : 4096 (six months). The blue white autofluorescence of theL. dumoffii colonies when viewed under ultraviolet light was particularly useful in preliminary identification. The patient recovered from his pneumonia after administration of erythromycin and rifampin. Legionella have been shown to multiply in monocytes and cell-mediated immunity appears to be the primary mechanism of host defense in man. Hairy cell leukemia is characterized by monocyte dysfunction and such patients have a predilection for infection by microbes that are controlled by cell-mediated defenses. We review other cases of community-acquiredL. dumoffii pneumonia as well as other cases of Legionella infection in patients with hairy cell leukemia.

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Nicht nosokomiale Pneumonie durch Legionella dumoffii bei einem Patienten mit Haarzell-Leukämie

Zusammenfassung Bei einem Patienten mit Haarzell-Leukämie wurde eine nicht nosokomialeLegionella dumoffii — Pneumonie beobachtet. Die Diagnose wurde gesichert durch kulturellen Erregernachweis aus Sputum und Bronchoalveolar-Lavageflüssigkeit, eine positive direkte Antikörper-Fluoreszenz und einen Antikörperanstieg im Serum mit einem Titer von 1 : 16 im Akutstadium auf 1 : 4096 nach sechs Monaten. Für die vorläufige Erregeridentifizierung war die weiße Autofluoreszenz derL. dumoffii-Kulturen unter ultraviolettem Licht besonders hilfreich. Nach Behandlung mit Erythromycin und Rifampicin heilte die Pneumonie ab. Es wurde nachgewiesen, daß sich Legionella in Monozyten vermehrt. Beim Menschen ist die zellvermittelte Immunität offensichtlich der primäre Abwehrmechanismus. Bei Haarzell-Leukämie besteht typischerweise eine Monozytenfunktionsstörung. Die Patienten sind besonders anfällig für Infektionen durch Erreger, die unter der Kontrolle der zellvermittelten Abwehr stehen. Wir geben eine Übersicht über andere Fälle vonL. dumoffii Pneumonie und andere Fälle von Legionella-Infektionen bei Patienten mit Haarzell-Leukämie.

     

Enhancement of granulopoiesis by lithium carbonate in a patient with hairy cell leukemia

A 73-year-old patient with hairy cell leukemia and profound granulocytopenia both before and after splenectomy was treated with lithium carbonate. With serum lithium level maintained in the therapeutic range, granulocyte count steadily increased from a level below 200/mm3 to 800/mm3 over a two-week period. This trend reversed upon withdrawal of lithium. A second hematologic response occurred when the drug was reintroduced. The data support the contention that lithium carbonate may effectively stimulate granulopoiesis on a long-term basis, and this pharmacologic approach may be a useful adjunct to the management of hairy cell leukemia.     

Anaplastic large cell lymphoma in a patient with rheumatoid arthritis

It is known that patients with rheumatoid arthritis (RA) have an increased risk for non-Hodgkin’s lymphomas in comparison with the general population. Although increased risk of lymphoma is attributed to the disease activity, the drugs used in the therapy of RA may also cause increased risk of malignancy. Herein, we report on an RA patient who developed non-Hodgkin’s lymphoma after methotrexate therapy and review the literature about it. A 74-year-old man with RA had been treated with low-dose methotrexate and subsequently developed anaplastic large cell lymphoma of the T-cell phenotype. Anaplastic large cell lymphoma has been reported rarely in rheumatoid arthritis.     

Rituximab: a useful drug for a repeatedly relapsed hairy cell leukemia patient

We describe a case of complete response in a patient with hairy cell leukemia, relapsed after treatment with interferon-alpha, 2'-deoxycoformicin, and 2-chlorodeoxyadenosine, and then successfully treated with rituximab. A fourfold reduction of leukemic cells was observed concomitantly with restoration of normal blood count and differential.     

Lipopolysaccharide responsiveness of malignant lymphoid cells in a patient with hairy cell leukemia.

The malignant cells in a patient with hairy cell leukemia responded most evidently to lipopolysaccharide (LPS) in in vitro culture for 3 1/2 days when the conventional tritiated thymidine uptake method was used. Since the malignant cells from patients with several other forms of leukemia and the peripheral blood mononuclear cells from healthy individuals did not show a comparable degree of responsiveness to LPS, we could exclude the possibility that this response was due to effects on contaminating normal mononuclear cells or to the nonspecific conditioning effect through LPS-affected contaminating normal monocytes. Morphological changes were observed with photo- and electronmicroscopy. It is likely that the hairy cells from the patient did respond to LPS, and whether or not this phenomenon may be confined to this type of lymphoid leukemia is not being investigated.     

Sequential development of large B cell lymphoma in a patient with peripheral T-cell lymphoma

Lymphomas of different histologic type can occur in the same patient. Two types of lymphomas can be diagnosed in the same lymph node (composite lymphoma) or in different sites. In the latter case, terms as simultaneous and sequential have been proposed to define the detection of two lymphomas at the same time or at different times, respectively.