18F-FDG PET/CT for Staging and Evaluation of Therapy in a Patient With Unusual Hairy Cell Leukemia Presentation.

Hairy cell leukemia is a rare hematologic malignancy characterized by splenomegaly, pancytopenia, and susceptibility to infections. We report a case of a 66-year-old man, diagnosed with hairy cell leukemia, without severe cytopenias and splenomegaly, but with an extensive pathological retroperitoneal mass and infiltration of the spleen and skeletal involvement. All findings were highly avid on pretreatment F-FDG PET/CT scan. Treatment response evaluation F-FDG PET/CT scan showed normalization of FDG uptake on all previously pathological sites.

Toxoplasmic Encephalitis with Untreated Hairy Cell Leukemia Variant.

Toxoplasmic encephalitis is a rare infectious complication in patients with hematological malignancy except for allogeneic hematopoietic stem cell transplantation (HSCT). We herein report a case of possible toxoplasmic encephalitis with untreated hairy cell leukemia variant. Magnetic resonance imaging showed multiple nodules with surrounding edema in the entire cerebrum. A polymerase chain reaction analysis for Toxoplasma gondii was negative. Her signs and symptoms fully recovered by empirical therapy with sulfadiazine and pyrimethamine. Toxoplasmic encephalitis may occur in patients who undergo non-allogeneic HSCT for hematological malignancies, even in those who have not been treated.

A case of hairy cell leukemia variant.

Hairy cell leukemia variant (HCLv) is a rare B-cell chronic lymphoproliferative disorder with features of the classic HCL but presenting some particularities, a poor response to conventional therapy of classic HCL and a more aggressive course of disease with shorter survival than classic HCL. We present a case of a 52-year-old man hospitalized in July 2012 in the Clinic of Hematology of Craiova, Romania, having splenomegaly, leukocytosis with lymphocytosis, anemia and thrombocytopenia, without monocytopenia, which exposed, in the peripheral blood and bone marrow cells, intermediate morphology between hairy cells and prolymphocytes and immunophenotype of mature B-cell phenotype CD19, CD20, CD22, CD11c, CD103, low positive for CD25 and negative for CD3, diagnosed with HCL variant, with no response to conventional chemotherapy and interferon-alpha, an aggressive course of disease and a survival of less than a year from diagnosis.

Unusual soft tissue infiltrates with 18F-FDG uptake in a patient with hairy cell leukemia.

Hairy cell leukemia (HCL) is an uncommon B-cell lymphoproliferative disorder, representing approximately 2% of leukemias. Diagnostic features include pancytopenia, splenomegaly, bone marrow reticulin fibrosis, and circulating hairy cells. Less commonly, there may be involvement of the liver and lymph nodes. We present a case of a 53-year-old man with HCL who was found to have soft tissue masses within the mediastinum and neck during pretreatment workup. An F-FDG PET/CT scan was requested to assess these lesions before treatment. These extensive infiltrates were FDG avid, and core biopsy of the mediastinal tissue was undertaken. Results were consistent with HCL.

Hairy cell leukemia in a patient with situs inversus totalis: an extremely rare combination.

Hairy cell leukemia is a rare cancer of the blood. The occurrence of hairy cell leukemia with another very rare genetic disorder makes us question whether it is just a coincidence. This article reports the first case of hairy cell leukemia in a patient with situs inversus totalis in western literature. There have been studies into the pathogenesis of situs inversus totalis that suggest it is caused by the failure of embryonic cells to properly rotate during embryogenesis. On the molecular level, the nodal cilia, which are responsible for embryonic rotation, are built by transport through the KIF3 complex - a kinesin superfamily of molecular motors. The KIF3 complex is also responsible for N-cadherin movement in cells. Furthermore, it is well known that these cell adhesion molecules play an important role in carcinogenesis and its progression. This report attempts to link the rare conditions and propose a possible genetic relationship between the two.

Hairy cell leukemia presenting as a peripancreatic mass: cytomorphology and radiographic correlates.

Hairy cell leukemia (HCL) usually presents with peripheral cytopenias, diffuse marrow infiltration, and splenomegaly. This chronic lymphoproliferative disorder is not typically associated with lymphadenopathy or mass lesions. We report a case of HCL first treated by splenectomy, followed by several years of interferon therapy. Twenty-five years later, the patient presented with weight loss, fatigue, and a large PET-avid mass surrounding the head of the pancreas. Fine-needle aspiration was pursued to investigate the unusual and infiltrative appearance of the lesion, which was suggestive of another primary malignancy. Cytology smears showed discohesive lymphoid cells with round nuclei and delicate cytoplasmic projections. Flow cytometry confirmed the presence of a clonal B-cell population with bright expression of CD20 as well as CD25 and CD103, diagnostic of HCL. This is the first report of HCL presenting as a peripancreatic mass. The importance of correlation with radiology and clinical history is emphasized when evaluating such lesions.

EUS-guided biopsy for the diagnosis and classification of lymphoma.

BACKGROUND: EUS-guided FNA and Tru-cut biopsy (TCB) is highly accurate in the diagnosis of lymphoma. Subclassification, however, may be difficult in low-grade non-Hodgkin lymphoma and Hodgkin lymphoma. OBJECTIVE: To determine the yield of EUS-guided biopsy to classify lymphoma based on the World Health Organization classification of tumors of hematopoietic lymphoid tissues. DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: A total of 24 patients referred for EUS-guided biopsy who had a final diagnosis of lymphoma or "highly suspicious for lymphoma." INTERVENTIONS: EUS-guided FNA and TCB combined with flow cytometry (FC) analysis. MAIN OUTCOMES MEASUREMENT: Lymphoma subclassification accuracy of EUS guided biopsy. RESULTS: Twenty-four patients were included in this study. Twenty-three patients underwent EUS-FNA, and 1 patient had only TCB. Twenty-two underwent EUS-TCB combined with FNA. EUS correctly diagnosed lymphoma in 19 out of 24 patients (79%), and subclassification was determined in 16 patients (66.6%). Flow cytometry correctly identified B-cell monoclonality in 95% (18 out of 19). In 1 patient diagnosed as having marginal-zone lymphoma by EUS-FNA/FC only, the diagnosis was changed to hairy cell leukemia after a bone marrow biopsy was obtained. EUS had a lower yield in nonlarge B-cell lymphoma (only 9 out of 15 cases [60%]) compared with large B-cell lymphoma (78%; P = .3 [Fisher exact test]). LIMITATIONS: Retrospective, small number of patients. CONCLUSION: EUS-guided biopsy has a lower yield to correctly classify Hodgkin lymphoma and low-grade lymphoma compared with high-grade diffuse large B-cell lymphoma.

Extranodal hairy cell leukemia presenting in the lumbar spine.

The authors report on a 54-year-old man who presented with a lumbar vertebral body lesion and an adjacent epidural lesion that was found to be hairy cell leukemia (HCL). The patient presented with gradual onset of back pain and intermittent lower-extremity radicular symptoms. He did not have splenomegaly or peripheral blood count abnormalities. Admission MR imaging revealed an L-5 vertebral body lesion and a lumbar epidural lesion extending from L-3 to S-2. An [18F]fluorodeoxyglucose-PET study showed numerous sites of osseous involvement. The patient underwent minimally invasive surgical biopsy sampling of the epidural lesion. Histopathological examination revealed extranodal HCL. After treatment with a 5-day course of cladribine, the patient's symptoms resolved, and at the 16-week follow-up visit there was no radiographic or metabolic evidence of disease. Hairy cell leukemia rarely involves neurological structures, but this patient responded well to standard treatment. This case demonstrates the value of tissue biopsy procedures instead of aggressive resection and the use of minimally invasive techniques to treat an HCL spinal lesion.

FDG-PET detection of primary bone marrow large B-cell lymphoma in a patient with hairy cell leukemia.

We describe a case of hairy cell leukaemia (HCL) coexistent with non-Hodgkin's lymphoma (NHD). This combination is reported to be extremely rare with no clear demonstration of the clonal relationship between the two conditions. After a previous failure of purine analogue therapy, our patient was successfully treated with rituximab resulting in normalisation of blood cell count cessation of blood transfusion and negative iliac crest biopsy. Unfortunately, the patient developed intense and persistent bone pain during the 1(st) line treatment for HCL. Skeletal X-rays, neck-thorax-abdomen CT scan and repeated bone MRI were unremarkable and bone scintigraphy showed non-specific changes. Laboratory examinations were normal. To better evaluate bone scintigraphy results, we finally performed FDG-PET/CT, which showed multiple foci of intense abnormal radiotracer uptake involving the bone marrow. An FDG-PET/CT guided bone marrow biopsy showed primary bone marrow diffuse large B-cell lymphoma (LBCL). Despite 2(nd) and 3(rd) line treatment, the patient died shortly after for central nervous system involvement by NHD. The role of FDG-PET/CT in identifying bone and bone marrow localization of NHD is reviewed and an earlier use is suggested in poorly understood bone pain.

Technetium-99m sulfur colloid scanning and correlative magnetic resonance imaging in patients with hairy cell leukemia and hypocellular bone marrow biopsies after 2-chlorodeoxyadenosine.

It has been observed that some patients in complete remission (CR) after 2-chlorodeoxyadenosine (2-CdA) for hairy cell leukemia (HCL) have hypocellular bone marrow biopsies despite normal peripheral blood cell counts. This discrepancy between bone marrow cellularity and peripheral blood cell counts suggests the possibility of abnormal sites of hematopoiesis. To determine sites of hematopoiesis, 11 radionuclide scans using technetium-99m (99mTc) sulfur colloid were performed in eight patients. Although no single, pattern was observed on the 99mTc sulfur colloid scans, two of the eight patients, both with virtually aplastic marrows, had multiple areas of increased uptake in the distal appendicular skeleton, suggesting abnormal sites of hematopoiesis. The same two patients had magnetic resonance imaging (MRI), which confirmed the abnormal sites of hematopoiesis.

Scintigraphic evaluation of the haemopoietic bone marrow using a 99mTc-anti-granulocyte antibody: a validation study with 52Fe.

To specify the validity of bone marrow scanning using a monoclonal anti-granulocyte antibody labelled with 99mTc (BW 250/183) for the functional assessment of haemopoiesis, we compared this method with 52Fe scan in 16 patients with haematological disorders. The examinations were performed using a rectilinear whole-body scanner and the distribution of the two tracers was assessed visually and quantitatively in anatomical bone marrow segments, the spleen and liver. Qualitative comparison showed concordance in the bone marrow distribution of the two tracers in 83% of the segments. Discrepancies were found in six patients with hypoplastic or aplastic marrow. The spleen was visualized in all cases with the 99mTc-Moab, including nine patients without splenic haemopoiesis (i.e. without spleen uptake of 52Fe). The uptake of the two tracers, quantified in bone marrow segments and the spleen, correlated well (P < 0.001), but not in the liver (NS). The correlation between the uptake values for each patient was excellent, except in cases of aplastic bone marrow. In conclusion, bone marrow scanning using a 99mTc labelled anti-granulocyte monoclonal antibody enables functional evaluation of the distribution of haemopoiesis. Limitations include the evaluation of bone marrow aplasia and identification of splenic haemopoiesis, for which 52Fe remains the tracer of choice.