Rajesh Kashyap

REDEFINING THE ROLE OF SPLENECTOMY IN PATIENTS WITH IDIOPATHIC SPLENOMEGALY

Background:  Despite extensive work‐up to establish the cause of splenomegaly, splenectomy may be required for diagnosis in certain situations. The aim of this study was to find out the role of diagnostic splenectomy in the current era.

Cranial nerve palsy in multiple myeloma and solitary plasmacytoma

Cranial nerve involvement in multiple myeloma and solitary plasmacytoma is rare. We report on two patients who developed cranial nerve palsy due to skull base plasmacytoma. Patient 1, a 55‐year old man with multiple myeloma, developed right sixth cranial nerve palsy during a phase of chemotherapy. He had an intracranial plasmacytoma in the clival region and was treated with 30 Gy whole brain radiotherapy. Patient 2, a 40‐year old man, presented with right third cranial nerve palsy and was detected to have a solitary skull base plasmacytoma. He was treated with radiotherapy followed by thalidomide plus dexamethasone.

Prevalence of autoimmune hemolytic anemia in multiple myeloma: A prospective study

Autoimmune hemolytic anemia (AIHA) is frequently associated with B‐cell lymphoproliferative disorders, and patients rarely develop overt clinical manifestations of AIHA. AIHA is rare in patients with multiple myeloma (MM). We conducted a prospective study to detect the presence of AIHA in MM patients and its impact on clinical presentation and outcome of the disease.

Splenic lymphoma with villous lymphocytes.

Splenic lymphoma with villous lymphocytes (SLVL) is a rare disorder that comprises less than 1% of lymphoid neoplasms. It is the leukemic counterpart of splenic marginal zone lymphoma (SMZL) and is characterized by splenomegaly, often with no lymphadenopathy, moderate lymphocytosis and villous lymphocytes on peripheral blood smear. Here, we report a case of SLVL in a 56-year-old male with very high leukocyte counts, massive splenomegaly and relatively few leukemic cells with subtle villous projections on the surface. This disorder is often confused with other chronic lymphoproliferative disorders, especially chronic lymphocytic leukemia (CLL) and hairy cell leukemia and should be differentiated from them. We are reporting this case to highlight the diagnostic pitfalls associated with this disorder.

T-cell prolymphocytic leukemia: a report of two cases with review of literature

T-cell Prolymphocytic Leukemia (T-PLL) is a mature post-thymic T-cell malignancy with aggressive clinical course. The principal disease characteristics are organomegaly, skin lesions and raised lymphocyte counts. We report two cases of T-PLL. T-PLL is a rare T-cell malignancy with characteristic clinical and laboratory features and a poor prognosis. It needs to be differentiated from B-Cell prolymphocytic leukemia (B-PLL) and other mature T-cell lymphoproliferative disorders with predominant leukemic pattern. Differentiation can be made by a comprehensive approach taking into account the clinical features, the cell morphology and the immunophenotype of leukemic cells.

Warm antibody hemolytic anemia-a rare presentation of neonatal lupus.

Neonatal lupus erythematosus (NLE) affects 1%–2% pregnant females with autoimmunity. An infant presented with steroid refractory hemolytic anemia as a manifestation of NLE. A trial of withholding breastfeeding had a transient response, but infant was eventually put on cyclosporin therapy to control the hemolysis. Now he is thriving well and transfusion free.

Hodgkin Lymphoma With Central Nervous System Disease at Primary Presentation

Letter to Editor: Pediatric Hodgkin lymphoma (HL) rarely presents with central nervous system (CNS) involvement at the time of initial presentation. Rather, it is more often seen in patients who have relapsed. We report a child with HL who had CNS involvement at initial presentation. A 6-year-old female presented with complaints of fever for 5 months, jaundice, and multiple nodular swellings in the neck and axilla for 3 months. On physical examination, she was febrile, pale, and icteric with cervical and axillary lymphadenopathy with largest lymph node 2 × 2 cm and massive hepatosplenomegaly. Laboratory investigations revealed anemia (hemoglobin 8.8 g/dl), total leucocyte count 5.2 × 109/l, platelet count 256 × 109/l, and abnormal liver function tests. Fine-needle aspiration cytology from the axillary node revealed scattered histiocytic clusters and atypical cells, with high nuclear–cytoplasmic ratio, hyperchromatic nuclei, and small nucleoli and the same was confirmed on biopsy. Immunohistochemistry displayed CD15 and CD30 positivity, which was consistent with the diagnosis of Hodgkin lymphoma, mixed cellularity type. Bone marrow biopsy showed infiltration with the tumor cells. Contrastenhanced computed tomography scan of the chest and abdomen revealed mediastinal and mesenteric lymphadenopathy along with hepatosplenomegaly. Considering Hodgkin lymphoma stage IVB she was started on gemcitabine, dexamethasone, cisplatin (GDP) chemotherapy regimen. On day 2 of chemotherapy, she had multiple episodes of generalized tonic clonic seizures that were associated with transient loss of vision in the postictal phase. Fundus and neurological examination remained normal and the patient child was managed with antiepileptics. Magnetic resonance imaging of the brain revealed multiple leptomeningeal deposits in the parieto-occipital and frontal region with altered signal intensity in corresponding regions (Fig. 1). Cerebrospinal fluid revealed pleocytosis with Reed–Sternberg cells. Chemotherapy was stopped, suspecting gemcitabine to be causative agent for loss of vision. She regained her vision after 5 days and remained seizure free. Thereafter, the chemotherapy protocol was changed. Radiotherapy was planned for CNS disease, but due to financial constraints, the child left against medical advice and could not be followed up. Hodgkin lymphoma involving the brain or meninges is uncommon and is usually seen in patients with uncontrolled systemic disease or with relapse.[1–5]. The median interval from initial diagnosis to intracranial disease presentation is almost 47 months.[2] Mostly, CNS HL shows a supratentorial spread, manifesting as cranial nerve palsies, motor or sensory deficits, headache, papilledema, coma, or seizures.[2,6] This case report highlights a rare clinical situation where a child developed CNS involvement in HL at the time of presentation. There are no definite guidelines for the management of CNSHL.Radiotherapywith or without systemic chemotherapy Fig. 1. T2-weighted image showing brainmagnetic resonance imaging (MRI) with enhancement of the parietooccipital region and corresponding leptomeningeal deposits.

Paroxysmal nocturnal hemoglobinuria clone in a case of myelodysplastic syndrome rapidly progressing to acute leukemia.

Myelodysplastic syndrome (MDS) comprises a group of stem cell disorders with considerable clinical and morphological heterogeneity. We report a case of MDS in a middle-aged male with clinical features of sepsis, dysplastic neutrophils and 6% blasts on bone marrow aspirate. A clone of neutrophils with deficient expression of CD16, CD55 and CD59 was found. A diagnosis of MDS with excess blasts with co-existent paroxysmal nocturnal hemoglobinuria (PNH) was made. Within a fortnight, the patient progressed to acute myeloid leukemia. We are reporting this unusual case of MDS displaying a sizeable clone of dysplastic neutrophils deficient in glycosyl phosphatidyl inositol anchored proteins, highlighting a common origin of PNH and leukemic clone.

Odontoid fracture in multiple myelomas

Fracture of the odontoid process as the primary presentation of multiple myelomas is rare. The management of this condition in an elderly patient presents a great challenge. We report a 60‐year‐old man who presented with fracture of the odontoid process of the C 2 vertebra with multiple myelomas.