Debdatta Basu

Clinico-hematological profile of Chediak-Higashi syndrome: experience from a tertiary care center in south India.

INTRODUCTION Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disorder characterized by partial ocular and cutaneous albinism, increased susceptibility to pyogenic infections, the presence of large lysosomal-like organelles in most granule-containing cells and a bleeding tendency. The abnormal granules are most readily seen in blood and marrow leukocytes, especially granulocytes; and in melanocytes. Other clinical features include silvery hair, photophobia, horizontal and rotatory nystagmus and hepatosplenomegaly. MATERIALS AND METHODS The clinico-hematological profile of a series of 5 cases of CHS encountered at JIPMER Hospital with diagnostic work-up done in the Department of Pathology over the last 6 years is presented. The diagnostic work-up included complete hemogram with peripheral smear, bone marrow examination, skin and liver biopsies. RESULTS The age of the patients ranged from 5 months to 3 years. All patients had silvery hair and partial albinism and presented with fever and recurrent chest infection. Two patients were stable. Three patients were in accelerated phase; of them, 1 patient with associated hemophagocytic syndrome had a rapidly fulminant course. Peripheral blood smear showed anomalously large granules in the leukocytes. Skin biopsy showed sparse, coarse melanin pigment in the epidermis, and liver biopsy done in 2 patients with accelerated phase showed portal lymphohistiocytic aggregates. CONCLUSIONS The diagnostic hallmark of CHS is the occurrence of giant inclusion bodies (granules) in the peripheral leukocyte and their bone marrow precursors. The case series is being presented because of the rarity of CHS and varied spectrum of clinical and hematological presentation.

Overview of blood components and their preparation

The whole blood which is a mixture of cells, colloids and crystalloids can be separated into different blood components namely packed red blood cell (PRBC) concentrate, platelet concentrate, fresh frozen plasma and cryoprecipitate. Each blood component is used for a different indication; thus the component separation has maximized the utility of one whole blood unit. Different components need different storage conditions and temperature requirements for therapeutic efficacy. A variety of equipments to maintain suitable ambient conditions during storage and transportation are in vogue. The blood components being foreign to a patient may produce adverse effects that may range from mild allergic manifestations to fatal reactions. Such reactions are usually caused by plasma proteins, leucocytes, red cell antigens, plasma and other pathogens. To avoid and reduce such complications, blood products are modified as leukoreduced products, irradiated products, volume reduced products, saline washed products and pathogen inactivated products. The maintenance of blood inventory forms a major concern of blood banking particularly of rare blood groups routinely and common blood groups during disasters. PRBCs can be stored for years using cryopreservation techniques. New researches in red cell cultures and blood substitutes herald new era in blood banking.

Sequential therapy versus standard triple drug therapy for eradication of Helicobacter pylori in patients with perforated duodenal ulcer following simple closure.

Abstract Background. Resistance to clarithromycin, a component of standard triple therapy, leads to inconsistent eradication rates of Helicobacter pylori infection. Some studies have shown higher eradication rates for H. pylori using sequential regimen. This study was done to compare the eradication rates for H. pylori infection between the standard triple drug therapy and the sequential therapy. Methods. Seventy-three patients with perforated duodenal ulcer following simple closure with H. pylori infection were randomized to receive either standard triple drug therapy or the sequential therapy. Standard triple drug therapy comprised of omeprazole, clarithromycin, and amoxicillin for 10 days. Sequential therapy comprised of omeprazole and amoxicillin or the first 5 days followed by omeprazole, clarithromycin, and amoxicillin for the next 5 days. Follow-up endoscopy was done at 2 months to assess the eradication rates, compliance, and side effects with each regimen. Results. Eradication rates for standard triple therapy and sequential regimen were 81.25% and 87.09%, respectively (p = 0.732). The cost of sequential therapy was cheaper and incidence of side effects and compliance were similar in each group. Conclusion. Standard triple therapy and sequential therapy have similar efficacy for eradication of H. pylori and sequential therapy is an economical alternative to standard triple therapy.

Immunohistochemical distinction between mesothelial and adenocarcinoma cells in serous effusions: A combination panel-based approach with a brief review of the literature

BACKGROUND The prognostic and therapeutic significance of differentiating adenocarcinoma (AC) from reactive mesothelium (RM) in effusions cannot be overemphasized. To avoid diagnostic errors, ancillary techniques like immunohistochemistry are employed. However, results vary and no universal standard has been accepted so far. OBJECTIVE To study the combined diagnostic efficacy of epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), E-cadherin (EC), calretinin (CAL), desmin (DES) and vimentin (VIM) in distinguishing RM from AC cells in serous effusions. STUDY DESIGN Unequivocally diagnosed cases of 39 adenocarcinomatous and 38 RM populations were studied using sections from 49 formalin-fixed, paraffin-embedded cell blocks. MATERIALS AND METHODS The immunomarkers were applied on cell block sections using the avidin-biotin peroxidase technique. The distribution/intensity of immunostaining in mesothelial and AC cells were graded semiquantitatively. STATISTICAL ANALYSIS USED Fischers exact test was used to calculate the efficacy of individual markers and their combinations. RESULTS EMA was the best single marker for AC, with 100% sensitivity and 97.37% specificity. For the mesothelial cells, CAL exhibited 100% sensitivity and 92.31% specificity. DES was more specific than CAL but had a poor sensitivity of 55.26%. EC, CEA and VIM had unsatisfactory predictive values precluding their use as individual diagnostic markers. Among the combinations, two panels--EMA+ AND (CAL- OR DES-) for ACs and CAL+ AND (EMA- OR CEA-) for RM had 100% specificities and sensitivities. CONCLUSIONS Most panel studies on fluid cytology are based on the arbitrary use of individual markers with the best statistical values, leading to a less than accurate diagnostic assessment. We believe that statistical parameters calculated in combination provide for a more practical and objective evaluation as well as allowing for meaningful comparative studies.

Pure neuritic leprosy with nerve abscess presenting as a cystic, soft tissue mass: Report of a case diagnosed by fine needle aspiration cytology

Pure neuritic leprosy (PNL) with nerve abscess manifesting as a huge, cystic, soft tissue mass is highly uncommon. Fine needle aspiration cytology can serve as an important initial diagnostic modality in such an instance.

Is enhanced platelet activation the missing link leading to increased cardiovascular risk in psoriasis

BACKGROUND Psoriasis is an immune mediated inflammatory skin disease associated with systemic inflammation resulting in increased risk for associated cardiovascular co-morbidities. The role of platelet activation in the pathophysiology of this condition has not been clearly studied. We undertook to study the platelet activation markers in psoriasis, as compared to controls and to identify its association with disease severity in psoriasis. METHODS Sixty-two patients with psoriasis and 62 age and gender matched healthy controls were enrolled in this cross-sectional study. The severity of the disease was assessed using the psoriasis area severity index (PASI) scoring. The platelet indices [mean platelet volume (MPV) and platelet distribution width (PDW)] were estimated by an automated haematological laser optical analyzer. Plasma soluble P-selectin and platelet derived microparticle (PDMP) concentrations, serum high sensitivity C-reactive protein (hs-CRP) and interleukin (IL)-6 concentrations were estimated in all study participants. Platelet aggregation was assessed using adenosine diphosphate (ADP) as aggregating agent. RESULTS We observed that there was significantly higher platelet indices (MPV and PDW) in patients with psoriasis, when compared to controls. Plasma soluble P-selectin concentrations, PDMP and platelet aggregation were significantly elevated in patients with psoriasis, as compared to controls. We also found significantly higher concentrations of hs-CRP and IL-6 in patients with psoriasis, as compared to controls. Platelet activation and systemic inflammation markers correlated positively with PASI, except PDW. We also observed significant positive correlation between platelet activation and systemic inflammation in psoriasis. CONCLUSION Significant platelet activation and systemic inflammation were observed in patients with psoriasis, especially when associated with severe disease. The increased platelet activation might be the missing link between the persistent inflammation and the development of atherosclerotic plaque leading onto cardiovascular co-morbidities seen associated with psoriasis.

Spectrum of histopathologic diagnosis of lymph node biopsies: a descriptive study from a tertiary care center in South India over 5½ years.

AIMS Lymphadenopathy is a common clinical problem and biopsies undertaken to determine the cause of nodal enlargement may be neoplastic or non-neoplastic. The former are mainly lymphohematogenous malignancies and metastases while the causes of non-neoplastic lymphadenopathy are varied. This study was undertaken to determine the histopathological spectrum of lymphadenectomies. MATERIALS AND METHODS This was a descriptive cross-sectional study wherein 1010 cases of histologically diagnosed peripheral lymph node biopsies in the Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry from January 2007 to June 2012 were reviewed. Surgical resection specimens with lymph node dissection were excluded from the study. RESULTS Neoplastic lesions were more common comprising 53% (535 cases) and included 32.1% (324 cases) of non-Hodgkin lymphoma, 12.4% (125 cases) of Hodgkin lymphoma and 8.5% (86 cases) of metastatic lesions. The non-neoplastic lesions were 47% (475 cases), which included 21.6% (218 cases) of non-specific reactive lymphoid hyperplasia, 6.8% (69 cases) of other reactive or specific lymphoid hyperplasia, 18% (182 cases) of tuberculous lymphadenitis, 0.6% (6 cases) of other granulomatous lesions. CONCLUSIONS Lymph node biopsy plays an important role in establishing the cause of lymphadenopathy. Among the biopsied nodes, lymphomas were the most common (44.5%) followed by non-specific reactive hyperplasia (21.6%), tuberculous lymphadenitis (18%) and metastasis (8.5%).

Fine Needle Aspiration Cytology of Oncocytic Lipoadenoma of the Parotid Gland

BACKGROUND Oncocytic lipoadenoma is an uncommon benign salivary gland tumor. There are only rare case reports of this distinct entity in which only the histopathologic aspects are discussed. Fine needle aspiration cytologic (FNAC) findings of oncocytic lipoadenoma have not yet been documented. CASE A 50-year-old woman presented with a slow-growing swelling in the left parotid region that was clinically interpreted as a soft tissue tumor, with a differential of neurofibroma/lipoma. FNAC showed moderate cellularity, with oncocytic cells arranged predominantly as microacini in a prominent lipoid background. The adipose tissue background of the cytologic smears was ignored as material derived from the normal fat tissue; based on the oncocytic population of cells, a diagnosis of oncocytoma was considered. A remote possibility of acinic cell carcinoma with oncocytic features was also suggested. However, histopathologic examination showed it to be an oncocytic lipoadenoma, a tumor we were unaware of at the time of cytodiagnosis. CONCLUSION Both cytopathologists and histopathologists need to be aware of oncocytic lipoadenoma of the salivary gland in order to diagnose it precisely. The clinicocytopathologic correlation highlighted in our case will be useful for cytopathologists in preoperative interpretation and diagnosis.

Significance of bone marrow fibrosis in multiple myeloma

Aims: The aim of the current study was to asses the frequency of increased fibrosis in myeloma and to find its correlation with other bone marrow parameters and survival. Methods: Forty‐four multiple myeloma patients diagnosed between 2001 and 2005 were included in the present study. A detailed study of the bone marrow aspiration smears and trephine biopsy was done. Bone marrow fibrosis was graded and correlated with other parameters like plasma cell morphology, pattern of infiltration, mitotic activity and also with the survival of the patients. Results: Increased fibrosis was seen in nine cases (20.5%). It was observed that plasma cell burden in the marrow was under‐estimated in the aspirate smears compared with the trephine biopsy in patients with increased fibrosis. Increased marrow fibrosis correlated significantly with poorly differentiated plasma cell morphology (p = 0.020) and mitotic activity (p = 0.003), which by themselves are established prognostic markers for survival in multiple myeloma. Patients with increased fibrosis of the marrow also had a median survival time of just 11 months. Conclusions: A bone marrow trephine biopsy is essential in all cases of myeloma at diagnosis, as it helps identify a subset of myeloma patients with increased marrow fibrosis and poorer prognosis.

Prognostic significance of bone marrow histology in multiple myeloma.

BACKGROUND Bone marrow examination continues to be the cornerstone for establishing the diagnosis of multiple myeloma in association with other clinical and laboratory parameters. Plasma cell morphology has significant correlation with clinical stage and survival. AIMS To note the bone marrow histology in detail in multiple myeloma and to correlate it with clinical stage and survival. METHODS AND MATERIAL Fifty-five cases of multiple myeloma diagnosed between January 2001 and December 2006, who had a bone marrow aspiration and biopsy done at the time of diagnosis were included in the present study. STATISTICAL ANALYSIS SPSS software version 13.0 was used. Clinical stage and plasma cell morphology were correlated using chi square test and Spearmans correlation coefficient. Survival analysis was done using the Kaplan-Meier method. RESULTS Seventy-six percent patients were in clinical stage III, 17% and 7% were in stage II and I respectively. The clinical stage correlated significantly with plasma cell morphology, percentage of plasma cell infiltration and pattern of infiltration. Plasma cell morphology correlated significantly with bone marrow parameters like percentage infiltrate, pattern of infiltration, degree of fibrosis and mitotic activity. Patients in advanced clinical stage,> 50% plasma cells in the marrow, diffuse pattern of infiltration, high mitosis and increased fibrosis had a shorter median survival than patients with favorable features. CONCLUSIONS It is recommended that the bone marrow histology be studied in detail in multiple myeloma at diagnosis since it correlates well with the clinical stage and offers useful prognostic information.