The May-Hegglin anomaly: a rare cause of a common complaint.

May-Hegglin anomaly is the presentation of a qualitative platelet disorder characterised by large platelets, thrombocytopenia and granulocyte inclusions as a result of mutations in the MYH9 gene. Though often overlooked given its rarity, it should be considered in patients who present with epistaxis, bruising, menorrhagia and easy bleeding as it can be mistaken for other diagnoses resulting in unnecessary treatments and tests. Our case study reports one presentation of this anomaly and can help broaden awareness of the presentation of this type of patient.

Chronic myelomonocytic leukemia evolving from preexisting myelodysplasia shares many features with de novo disease.

The majority of chronic myelomonocytic leukemia (CMML) cases arise de novo; cases evolving from preexisting myelodysplasia (MDS) or myeloproliferative diseases have not been well-studied. We conducted the present study to determine the clinicopathologic features and to study possible underlying molecular and cytogenetic mechanisms involved in this evolution. Between April 1995 and November 2005, we identified 120 CMML cases, of which 20 (16.7%) had a previous diagnosis of MDS. Of the 20 patients with MDS, 6 had relative monocytosis at diagnosis. At the time of MDS to CMML evolution, mutations in JAK2 (V617F), FLT3 (ITD), K-ras-2, or N-ras were not acquired, and only 1 (6%) of 17 evaluable cases showed cytogenetic progression. The median time to evolution from MDS to CMML was 29 months, and the median survival following CMML development was 13 months. Three cases (17%) transformed to acute myeloid leukemia. These findings indicate that in some cases of otherwise typical MDS, the progenitor cells may have some capacity for monocytic proliferation at diagnosis and manifest rapid disease progression once a monocytic proliferation supervenes.

Cytological features of Castleman disease: a review.

INTRODUCTION: Castleman disease (CD) is a benign lymphoproliferative disorder with hyaline vascular (HVCD), plasma cell (PC-CD), and mixed subtypes. Only HVCD lymph node cytomorphology has been described, mainly as case reports. We reviewed all CD subtypes. To the best of our knowledge, our case series is the largest and most comprehensive yet published. MATERIALS AND METHODS: We searched our institution's database for histologically confirmed CD cytology cases (fine needle aspiration, touch preps) for the past 23 years. Two independent pathologists evaluated cytomorphology. We then reviewed touch preps from 6 histologically confirmed, non-CD reactive lymph node excisions. RESULTS: 8 patients (5 women, 3 men) had the following subtypes: HVCD (5 patients), PC-CD (2), and mixed (1). All cases had a heterogenous background population composed predominantly of small lymphocytes with single and clustered follicular dendritic cells (FDCs). The FDCs had delicate pale cytoplasm with indistinct borders showing lymphocyte emperipolesis. They were often binucleated or multinucleated with fine chromatin, regular nuclear borders, large nuclei, and small nucleoli. HVCD cases had traversing, frequently hyalinized capillaries. PC-CD cases had increased plasma cells, including binucleate forms, and tingible body macrophages with fewer FDC clusters. Human herpes virus-8 immunostain was negative in all cases. Non-specific follicular hyperplasia cases had abundant tingible body macrophages, rare hyalinized capillaries, and no lymphocyte emperipolesis. CONCLUSIONS: CD is distinguished by background lymphocytes and cohesive FDC clusters with lymphocyte emperipolesis. HVCD has traversing, hyalinized capillaries and PC-CD has increased plasma cells and tingible body macrophages. Knowledge of these features can prevent a lymphoma misdiagnosis.

Post-transplant lymphoproliferative disorder of the cervical spine mimicking an epidural abscess.

Post-transplant lymphoproliferative disease (PTLD) is a recognized complication following solid organ and stem cell transplants with subsequent immunosuppression and is the most common malignancy complicating solid organ transplantation. Improved survival and use of aggressive immunosuppression following solid organ transplants have led to increased diagnosis of PTLD. Nevertheless, spinal involvement in PTLD is extremely rare. To our knowledge, this is the first report of PTLD causing epidural spinal cord compression of the cervical spine, mimicking the imaging and pathology of an epidural abscess. The patient underwent posterior and subsequent anterior decompression and stabilization. Rarity of occurrence of PTLD in the spine with absence of diagnostic imaging features may preclude differentiating it from the more commonly occurring lesions such as epidural abscess which occurs in a similar clinical setting. As the management strategy and overall prognosis are dramatically different, the importance of considering PTLD in the differential diagnosis for epidural spinal cord compression in a transplant recipient patient cannot be overemphasized.

Kikuchi-Fujimoto disease: hydroxychloroquine as a treatment.

We describe a case of recurrent Kikuchi's disease in a South Asian-American man that was treated successfully with chloroquine and on recurrence with hydroxychloroquine. Each treatment led to a very prompt response.

Sideroblastic anemia due to linezolid in a patient with a left ventricular assist device.

An acquired form of sideroblastic anemia has been described in association with several drugs, especially anti-microbial agents. A case of sideroblastic anemia is presented in a patient with a left ventricular assist device drive-line infection who was receiving linezolid, an antibiotic used for serious infections with gram-positive organisms. This patient's anemia resolved after discontinuation of the drug; he subsequently underwent an uncomplicated orthotopic heart transplant with no recurrence of anemia. As linezolid has been shown to have hematologic side effects, blood count monitoring is recommended in patients receiving this drug for long-term therapy.

Fine-needle aspiration of subcutaneous panniculitis-like T-cell lymphoma.

We report the fine-needle aspiration (FNA) cytology findings of Subcutaneous Panniculitis-like T-cell Lymphoma (SCPTCL) in a 66-year-old woman who presented with a four month history of asymptomatic subcutaneous nodules on her right chest wall and back. An excisional biopsy of the right chest nodules was performed, and the diagnosis of SCPTCL was rendered. On a follow-up visit, several skin lesions were noted throughout her body. A fine-needle aspiration (FNA) of the right inguinal region was performed. The FNA yielded cellular smears, composed mainly of sheets of epithelioid histiocytes and scattered multinucleated cells. However, no distinct granulomas were noted. The background of the cytological smears showed scattered atypical lymphoid cells, some of which displayed nuclear membrane irregularities. To the best of our knowledge, the cytological features on FNA material of SCPTCL have not been described.

Anaplastic T large cell lymphoma diagnosed by exfoliative cytology in a post renal transplant patient.

In the last two decades posttransplant lymphoproliferative disorders (PTLDs) have been recognized as a complication of organ transplantation with immunosuppression. The reported incidence of PTLDs in renal transplant patients ranges between 0.3-3% (Birkeland et al., Transplantation 1999;67:876-881). In contrast to the reported incidence of PTLDs in post bone marrow transplant, it is 1% in HLA-matched recipients and up to 20% in HLA mismatched T-cell depleted bone marrow recipients (Curtis et al., Blood 1996;94:2208-2216). In cardiac transplant recipients the reported incidence of PTLDs is between 1.8-9.8 (Mihalov et al., Clin Transplant 1996;10:248-255). PTLDs are predominately extranodal. They have varied morphologic patterns and clonality, but almost all are associated with Epstein-Barr virus (EBV). The vast majority are of B cell lineage; only about 10% are of T-cell origin. We report a T-cell anaplastic large cell lymphoma (ALCL) presenting with bilateral pleural effusion and liver involvement in a renal transplant recipient.

Pentoxifylline, Ciprofloxacin and Dexamethasone Improve the Ineffective Hematopoiesis in Myelodysplastic Syndrome Patients; Malignancy.

Twenty-five patients with a diagnosis of myelodysplastic syndromes (MDS) were randomized to either begin therapy with pentoxifylline, ciprofloxacin and dexamethasone (PCD) immediately (10 patients) or after a 12 week observation period (control arm, 15 patients). PCD was administered with the goal of suppressing cytokine-induced excessive intramedullary apoptosis of hematopoietic cells. No marked fluctuations of blood counts were noted during the period of observation. Twenty-two patients completed at least 12 weeks of therapy: 18/22 showed some type of hematologic response, 9/18 showing an improvement in absolute neutrophil count only (p = < 0.001) and 9/18 showing multi-lineage responses. No unique category of MDS responded better, however 19/25 patients had refractory anemia (RA)/RA with ringed sideroblasts. The median time to response was 6 weeks and 3/18 responding patients maintained their responses beyond a year. We conclude that hematologic improvement in response to PCD therapy supports the validity of this unique anti-cytokine approach. Future trials should combine PCD therapy with established approaches (growth factors/chemotherapy) and also should focus on identifying more effective ways of suppressing the pro-apoptotic cytokines in MDS.