Sonal Jain

Pantoea agglomerans infection behaving like a tumor after plant thorn injury: An unusual presentation

Pantoea agglomerans infections in humans are uncommon. Most common infections reported are septic arthritis or synovitis. We report the case of a 25-year-old, healthy male, who presented with indurated swelling over the posterolateral aspect of his right thigh, associated with pain for one month. Magnetic resonance imaging (MRI) revealed muscle edema with cystic areas in the posterior-most part of the vastus lateralis of the right thigh. The condition was clinically diagnosed as a right-sided benign tumor of the vastus lateralis muscle. However, Pantoea agglomerans was isolated on a culture of the excised muscle tissue. On the basis of the awareness of the common association of Pantoea with penetrating trauma by vegetation, the patient was asked to recollect any prior such injury. He then gave a history of a fall in the field and a plant thorn prick in the thigh four years back, when he was an agricultural worker. We emphasize the importance of Pantoea agglomerans infection of the soft tissues that can have an atypical presentation as a non-suppurative, indurated, muscle cyst in our case. Thorn injuries are usually ignored as trivial incidents, however, Pantoea infections should always be borne in mind when encountering soft tissue lesions, as antibiotic treatment is required for complete resolution of the lesion.

Aberrant Immunophenotypic Expression of CD5 in a Case of B Acute Lymphoblastic Leukemia: A Case Report

Abstract Aberrant expression of CD5 has rarely been reported in B cell lineage acute lymphoblastic leukemia (B-ALL). We report the rare immunophenotypic expression of CD5 in a 20-year-old male who was diagnosed to have B-ALL on bone marrow examination. Cytogenetic analysis revealed a mosaic supernumerary marker chromosome. The patient died due to acute pancreatitis after admission. CD5 positive B-ALL may represent a distinct clinicopathologic subcategory of B-ALL with an aggressive clinical course.

Flow Cytometry in Hematological Disorders

Flow cytometry with its rapidly increasing applications is being used essentially in all fields of diagnostic medicine. In hematological disorders it is most commonly used in diagnosis, characterization, prognostication and even selecting target therapy of acute leukemia and to some extent lymphomas. It is increasingly finding place in other fields of hematology i.e., non-malignant disorders of all blood cell types including RBCs and platelets along with leukocytes. In this review the authors have discussed some of these applications with an emphasis on disorders specific to pediatric patients.

Evaluating New Markers for Minimal Residual Disease Analysis by Flow Cytometry in Precursor B Lymphoblastic Leukemia

Minimal residual disease is currently the most powerful prognostic indicator in Precursor B lymphoblastic leukemia. Multiparameter flow cytometry is the most commonly used modality. Seventy three B ALL cases and 15 normal marrows were evaluated for expression patterns of leukemia markers (CD38, CD58, CD73) in all 73 cases and CD66c, CD86 and CD123 in 23 cases. CD73 was aberrantly expressed in 90.41% cases and CD86 in 60.87% B ALL cases. Thus addition of these markers in MRD panels can increase the sensitivity of the assay.

Precursor NK Cell Lymphoblastic Leukemia/Lymphoma—Report of a Case with Literature Review

Abstract Precursor Natural Killer (NK) cell lymphoblastic leukemia/lymphoma is a rare entity defined clearly by WHO (2008 WHO classification). However, the pathobiology of this subset of neoplasms is not clearly defined. There is wide disparity in the literature regarding the nomenclature and diagnostic criteria used to diagnose and characterize acute leukemias of presumed NK cell origin. In the present article we report a case of Precursor NK cell lymphoblastic leukemia/lymphoma and review the cases reported after 2008 WHO classification came into vogue, as acute leukemias of NK cell origin.

Chronic myeloid leukemia with p210 BCR–ABL and monocytosis

Chronic myeloid leukemia (CML) is characterized by splenomegaly, a high total leukocyte count, and cytomorphological features of granulocytic hyperplasia. The diagnosis is established by demonstration of the BCR–ABL fusion transcript by reverse transcriptase-polymerase chain reaction (RT-PCR). Monocytosis is, however, an uncommon feature of CML at presentation [1]. Here, we report a rare case of adult CML with monocytosis. A 37-year-old male presented with splenomegaly of two and a half years duration. On examination he was found to have hepatosplenomegaly. A complete hemogram showed anemia and a high total leukocyte count (hemoglobin 10 g%, total leukocyte count 19.6610/L, platelet count 200610/L). A differential leukocyte count on a peripheral smear showed 23% mature monocytes (Bl01Promono01My02 MMy08N46 L08M23E06B05) with an absolute monocyte count of 4.5 6 10/L [Figure 1(A)]. There were no dysplastic changes on the peripheral smear. Bone marrow aspiration smears were cellular and showed myeloid preponderance with left shift, with mature monocytes constituting 15%. Blasts and promonocytes constituted 13% (myelogram: Bl08Promono05My10MMy14N30L06M15E01B03NRBC08; myeloid:erythroid [M:E] ratio, 10.7:1). There was significant megakaryocytic dysplasia with megaloblastic change in the erythroid series and no significant dysplasia in the myeloid series. In view of the high absolute monocyte count in the peripheral blood (416 10/L), a diagnosis of chronic myelomonocytic leukemia (CMML) was considered, and RT-PCR for BCR– ABL and leukocyte alkaline phosphatase (LAP) test were advised. The LAP score was low (test 16, control 162). RT-PCR for BCR–ABL showed the p210 fusion transcript [Figure 1(B)]. Hence, the case was diagnosed as CML in accelerated phase. Chronic myeloid leukemia is associated with the Philadelphia chromosome t(9;22)(q34;q11), which results in the BCR–ABL fusion transcript. There can be three fusion transcripts based on the breakpoint in the BCR gene on chromosome 22: p210, p190, and p230 [2]. Monocytosis in CML is rare at presentation. The association of monocytosis with p190 fusion transcript-positive CML was described in 1994 by Melo et al. The authors suggested that this could be a missing link between CMML and CML, with morphological features between the two [3]. This association of monocytosis with p190 CML has been in seen in a few cases reported in the literature [4–6]. Verma et al. reported that p190 BCR–ABL fusion protein is associated with the minor breakpoint cluster region in chromosome 22, and is seen in *1% of cases of CML [5]. In their series, five out of nine patients with CML in chronic phase had monocytosis. This subtype has been associated with a poor response to tyrosine kinase inhibitors [5]. However, most cases of CML have a breakpoint in the major breakpoint cluster region (M-bcr) including introns 13 and 14, resulting in the 210 kDa (p210) fusion transcript [2]. In the present case, monocytosis was associated with the classic p210 BCR–ABL transcript. Two pediatric cases of CML with p210 in association with monocytosis have previously been reported [7]. One patient was a 19month-old boy and the other was a 9-month-old boy, both of whom were suspected to have juvenile myelomonocytic leukemia (JMML). However, this association has not been reported in adult cases of CML.

Coexistent Multiple Myeloma and SLL: A Rare Entity

A 64 years old male, presented to the hospital with complaints of lower back pain for 1 month. On examination, patient had a 2 9 2 cm submandibular lymph node and a 1 9 1 cm left axillary lymph node. CBC with peripheral smear examination showed rouleaux formation and increase in small lymphocytes (DLC: N: 41, L:55, M:04). MRI of the dorso-lumbar spine revealed multiple altered signal intensity lesions in the dorsolumbar spine, pelvis and ribs, associated with soft tissue component favoring a possibility of neoplastic etiology? Metastasis. Bone marrow aspiration (BMA) and bone marrow biopsy (BMB) were undertaken. BMA smears showed increase in plasma cells showing abnormal morphology along with an increase in small lymphoid cells (Myelogram: My:05, N:11, L:40, M:03, E:04, Erythroid:20, Plasma Cells: 17). BMB showed 6–7 lymphoid aggregates which were predominantly intertrabecular in location and comprised of small lymphoid cells with monomorphic appearance. Adjacent to these aggregates, clusters of plasma cells were found (Fig. 1). On IHC: These lymphoid cells were positive for CD20 highlighting the ‘‘B’’ nature of these cells. These cells were also positive for CD5, CD23, Bcl2 and negative for CYCLIN D1, CD10, CD38, CD138, CD3 and showed ki67 index of \5 % (Fig. 2). CD38 AND CD138 highlighted multiple clusters and aggregates of plasma cells lying throughout the section examined These plasma cells expressed lambda and were negative for kappa and CD20 (Fig. 3). Serum protein electrophoresis done subsequently showed M spike of 3.5 g/ dl. On the basis of these findings, a final diagnosis of multiple myeloma coexistent with bone marrow infiltration by SLL was rendered. The differential diagnosis of a lymphoplasmacytic/lymphoplasmacytoid infiltration in the bone marrow include (1) Lymphoplasmacytic lymphoma, (2) Marginal zone lymphoma with plasmacytic differentiation, and (3) CLL with plasmacytic differentiation [1]. Lypmhoplasmacytic lymphoma presents with admixture of plasma cells, plasmacytoid lymphocytes and lymphocytes in the bone marrow, however completely separate lymphoid and plasma cell collection may occur occasionally. The lymphocytes of LPL are CD5 negative [2]. 21–74 % of splenic marginal zone lymphoma show plasmacytic differentiation. These cells can be CD5? in up to 20 % cases but are strong CD20 and surface IgG? thereby differentiating it from CLL [1]. CLL itself can show lymphoplasmacytoid differentiation, but such cases will lack true plasma cells. The small lymphocytes would be CD5 and CD23? and would show dim CD20 and surface IgG expression. Presence of end organ damage, lytic bone lesions, non IgM paraprotein support a diagnosis of MM because such features are rare in these lymphoid malignancies. The correct distinction of the disease as concomitant MM and CLL from other causes of marrow lymphopasmacytoid infiltration is very important for the initiation of correct therapy of the patient. & Sonal Jain sonalmalhotra80@gmail.com

CD34 immunohistochemistry in bone marrow biopsies for early response assessment in acute myeloid leukemia

Acute myeloid leukemia is a heterogenous disease with respect to prognosis. Early response assessment has an established role as predictor of remission rate, and overall and disease‐free survival. Assessment of blast percentage on bone marrow aspirate smears at this stage has its own limitations.

An unusual clonal cytogenetic abnormality with t(15;17)(p11;q21) in a patient with severe aplastic anemia.

Aplastic anemia is a rare, serious disease characterized by pancytopenia and hypocellular bone marrow. We present a case of severe aplastic anemia with a novel cytogenetic abnormality involving a balanced translocation between chromosomes 15p11 and 17q21. The breakpoint in chromosome 17q21 was similar to that involved in acute promyelocytic leukemia. A 68-yearold male presented with complaints of progressively increasing weakness and low-grade fever for 30 days. There was no bleeding from any site. Past medical history and family history were non-contributory. Clinical evaluation revealed palor with no lymphadenopathy or organomegaly. Hematological workup revealed pancytopenia with hemoglobin 6.8 g/dl, total leukocyte count 1.2 × 109/l, absolute neutrophil count 0.24 × 109/l, platelet count 26 × 109/l, and peripheral blood differential count with neutrophils 20% and lymphocytes 80%. Bone marrow biopsy revealed hypocellular marrow with overall cellularity less than 5%. There was no marrow dysplasia, tumor infi ltration, or myelofi brosis. Investigations for viral infections like hepatitis B virus, hepatitis C virus, human immunodefi ciency virus, and parvovirus B19 were negative. Chromosomal analysis revealed a male karyotype with the presence of translocation between chromosome 15 and 17 [46 XY, t(15;17)(p11;q21)] in all metaphases, detectable at the level of banding resolution (ISCN) 400. Paroxysmal nocturnal hemoglobinuria clone was ruled out by fl owcytometry on peripheral blood granulocytes. Prothrombin time and activated partial thromboplastin time were normal with no evidence of coagulopathy. He was given supportive treatment with packed red blood cell transfusion and started on oral cyclosporine 100 mg twice daily. Most cases of aplastic anemia are acquired and do not have any cytogenetic abnormalities though few patients have been described with these abnormalities at presentation ranging from 4% to 12%.[1-4] The relevance of cytogenetic abnormalities to the pathophysiology of AA is unknown in contrast to diseases like myelodysplastic syndrome and acute leukemias where diagnosis and treatment rests heavily on cytogenetic abnormalities. Both numerical and structural abnormalities have been reported in aplastic anemia. Numerical abnormalities include trisomies of chromosome 6, 7, 8, 13, 14 and 15 and monosomy 7 and 9 and structural abnormalities include t(3;11) and t(4;6).[1,2,4] Overall, the most common chromosomal abnormalities reported are trisomies of 6 and 8 and loss of chromosome 7.[1,4] Although unusual cytogenetics have been reported in patients with AA[5,6] including t(9;22) but t(15;17) has not been reported. The response to immunosuppressive therapy, durability of response, and progression to later clonal disorders in these patients did not appear to be different from patients with a normal karyotype though they might be at higher risk of progressing to myelodysplastic syndrome or acute myeloid leukemia.[2,4]