Reversal of life-threatening bleeding with protamine sulfate in a patient with plasma cell leukemia.

Plasma cell disorders, such as multiple myeloma, can cause numerous derangements of hemostasis. In this case report, we present a life-threatening coagulopathy in a patient with progressing multiple myeloma in which the antibody-producing heparin-like activity is a free light chain. The patient's bleeding was successfully treated using protamine sulfate, which then allowed treatment of her plasma cell leukemia. In the literature, other authors have reported similar patients who have responded to protamine sulphate either in vitro or in vivo , providing further evidence for the role of protamine sulfate in the reversal of coagulopathy and resolution of bleeding diathesis. Standard treatments of transfusion with fresh frozen plasma and cryoprecipitate are likely to be ineffective in life-threatening bleeding related to this mechanism (heparin-like effect), and it is essential that treating physicians are aware of this potential mechanism of bleeding in their patients.

[Transudative myelomatous pleurisy revealing pleural localization of primary plasma cell leukemia]. / Pleurésie myélomateuse transudative révélant une localisation pleurale d'une leucémie à plasmocytes de novo.

Primary plasma cell leukemia (PPCL) is an aggressive and rare variant of multiple myeloma (MM), with frequent extramedullary involvement, mainly liver and splenic lesions. Pleuropulmonary involvement has rarely been described in the literature. We report a case of primary plasma cell leukemia in a 46-year-old patient, whose first symptom was pleural effusion with mediastinal adenopathies simulating a pleural localization of a lymphomatous process. However, blood smear examination, electrophoresis as well as immunofixation of plasma proteins and immuno-histochemistry have helped to guide the diagnosis. Pleurisy is a rare mode of revelation of plasma cell leukemia and is a factor of poor prognosis.

Blastic plasmacytoid dendritic cell neoplasm: a rare case of gingival lesion with leukaemic presentation.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy with an aggressive clinical course. It has been recognised as a distinct entity in the WHO 2008 classification of haematolymphoid neoplasm. This disease usually presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood with leukaemic dissemination. Gingival lesion as the first manifestation, in the absence of a cutaneous lesion, is an uncommon presentation of this rare disease. We report a case of an elderly woman, aged 84 years, who presented with a lump on the mandibular gingiva and ipsilateral otalgia without any cutaneous lesion and associated with an highly aggressive and rapid leukaemia. This case is an addition to the handful of those cases of BPDCN which presents without cutaneous involvement, but it is exceedingly rare for clinical features that make it a unique case in the literature.

A Woman in Her 40s With Headache and New-Onset Seizures.

A woman in her 40s with a history of plasma cell leukemia presented with 1 month of intermittent headaches followed by a seizure. Results from laboratory studies were notable for a cerebrospinal fluid opening pressure of 28 mm H2O and 8 white blood cells, including 1 atypical plasma cell. Imaging studies revealed confluent bifrontal white matter fluid-attenuated inversion recovery hyperintensities, as well as a contrast-enhancing sellar lesion. The patient underwent a stereotactic biopsy. The differential diagnosis, pathologic findings, and diagnosis are discussed.

Multiple myeloma and secondary plasma cell leukemia.

Plasma cell leukemia is very rare condition characterized by malignant proliferation of plasma cells in blood and bone marrow, which is aggressive and has a short survival even with conventional treatment. This ominous entity may be primary, or develops secondarily during the course of multiple myeloma. A 53-year-old Brazilian woman with multiple myeloma is described with bone marrow evaluation revealing 25% plasma cells. The quantification of plasma cell infiltration in bone marrow aspirate and immunohistochemistry study revealed consistent features of myeloma and plasma cell leukemia, and lambda light chain expression. Worthy of note was the absence of CD56 expression and the expression of CD20; moreover, 23% of circulating plasma cells were detected in peripheral blood smears. Therefore, the diagnosis of plasma cell leukemia was characterized and therapeutic schedules with dexamethasone, thalidomide, cisplatin, doxorubicin, cyclophosphamide, and etoposide were utilized. With significant clinical improvement, the patient is currently waiting for bone marrow transplant.

Rapid Fatal Acute Peripheral T-Cell Lymphoma Associated With IgG Plasma Cell Leukemia and IgA Hypergammaglobulinemia.

Simultaneous occurrence of T-cell and B-cell neoplasms is rare, and etiologic relationships between these 2 malignancies are poorly understood. We describe the case of a 66-year-old woman who was admitted to the hospital because of fever, hemoptysis, lymphadenopathy, and skin rash. Enlarged lymph nodes in axillary, pectoral, paratracheal, and periportal regions as well as slight hepatomegaly and splenomegaly were confirmed. A peripheral blood smear revealed rouleaux formation and numerous circulating plasma cells, with plasmacytoid lymphocytes. Immunofixation-electrophoresis detected a monoclonal band defined as immunoglobulin (IgG)-lambda light chains with broad-band polyclonal IgA. The patient died from abrupt splenic rupture before diagnostic work-up was finished. Postmortem examination revealed infiltration of atypical lymphoid cells exhibiting high proliferative activity admixed with typical and atypical plasma cells in several organs. Thus, plasma cell leukemia (IgG-lambda) as a rare and aggressive variant of plasma cell myeloma in the present case was associated with aggressive peripheral T-cell lymphoma and polyclonal (IgA) plasmacytosis.

Primary plasma cell leukemia: A report of two cases of a rare and aggressive variant of plasma cell myeloma with the review of literature.

Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma accounting for 2-3% of all plasma cell dyscrasias characterized by the presence of circulating plasma cells. The diagnosis is based on the % (≥20%) and absolute number (≥2x10 9 /L) of plasma cells in the peripheral blood. The incidence of primary PCL (pPCL) is very rare and reported to occur in <1 in a million. It is classified as either pPCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. pPCL is a distinct clinicopathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. We report two cases of pPCL, both having acute onset of illness, varied clinical presentation with one of them showing "hairy cell morphology," with rapidly progressing renal failure, and was not suspected to be plasma cell dyscrasia clinically. A detailed hematopathological evaluation clinched the diagnosis in this case. It is recommended that techniques such as immunophenotyping by flow cytometry and protein electrophoresis must be performed for confirmatory diagnosis. A detailed report of two cases and a review of PCL are presented here.

Abrupt Development of Plasma Cell Leukemia in a Patient with Chronic Anemia under Follow-up.

Plasma cell leukemia (PCL) is a very rare and progressive hematologic malignancy with unpleasant prognosis, which present with monoclonal proliferation of plasma cells in peripheral blood. Here we report a 52-year-old female case of PCL which diagnosed by morphology and immunohistochemistery (IHC) study. IHC revealed CD20+/CD38+/CD138+/CD56-/kappa-/lambda+. We diagnose PCL on peripheral blood. IHC can be helpful for prognostic determination.

Simultaneous Occurrence of Angioimmunoblastic T-cell Lymphoma and Plasma Cell Leukemia

No abstract available.

Plasma cell leukaemia a rare cause of disproportionate anaemia in a patient presenting as CKD.

The anaemia in patient of chronic kidney disease is commonly related to secondary erythropoietin deficiency. When the severity of anaemia is disproportionate and associated with other haematological abnormalities like thrombocytopenia, then primary haematological disorder and secondary renal involvement must be considered. Renal involvement is common in haematologic disorder like Multiple Myeloma. The diagnosis of haematological disorder may be missed. This is a case of chronic kidney disease with disproportionate severe anaemia with bleeding diasthesis which ultimately turned out to be plasma cell leukaemia.