Classification of malignant lymphoma subtypes in Korean patients: a report of the 4th nationwide study Abstract To determine the relative frequency and change of malignant lymphoma in Korea according to the 4th World Health Organization (WHO) classification and compare with previous reports. Between 2015 and 2016, 7737 new patients with malignant lymphoma were enrolled from 31 institutes, with their clinicopathologic information obtained, and evaluated for the relative frequency of lymphoma subtypes. The relative frequency of non-Hodgkin lymphoma (NHL) was 94.8%, and that of Hodgkin lymphoma (HL) was 5.2%. B cell lymphomas accounted for 83.1% of all NHLs; T/natural killer (NK) cell lymphomas, 16.4%; and immunodeficiency-associated lymphoproliferative disorders, 0.5%. The most common NHL subtypes were diffuse large B cell (41.5%), extranodal marginal zone (MALT, 19.8%), follicular (7.5%), NK/ T cell (4.2%), and peripheral T cell lymphomas, not otherwise specific (PTCL, NOS, 3.4%). Nodular sclerosis was the predominant HL subtype (48.5%), followed by mixed cellularity (28.7%), lymphocyte-rich (6.8%), lymphocyte-depleted (1.5%), lymphocyte-predominant (2.8%), and unclassified HL (11.8%). Compared with a previous report, increased B cell lymphomas (77.6–83.1%) and slightly decreased NK/T cell lymphomas and PTCL were observed. The incidence of follicular lymphoma increased by more than 2.5-fold (2.9–7.5%). Incidence rates of newly diagnosed lymphomas were lower for HL and higher for extranodal NHL, MALT, and nasal type NK/T cell lymphomas in Korea than those in Western countries. A slight increase in the relative frequency of B cell lymphoma and a prominent increase in follicular lymphoma may be attributed to refined diagnostic criteria and Westernized disease patterns.

Platelet satellitism: unusual cause of spurious thrombocytopenia Abstract Platelet satellitism (PS) is defined as adherence of platelets on the surface of polymorphonuclear leucocytes and imparting a rosette-like appearance especially in ethylenediaminetetraacetic acid (EDTA) whole blood samples. We here present a case of spurious thrombocytopenia resulting from florid platelet adherence on the surface of neutrophils.

SOLAHP: a fledgling organization rapidly taking flight

Association between serum levels of hepcidin and ferritin in patients with thalassemia major and intermedia, the role of iron chelator Abstract Patients with beta-thalassemia suffer from iron overload, and hepcidin, as the main regulator of iron hemostasis, can indicate iron overload better than serum ferritin, but changes in serum hepcidin levels are affected by the type of thalassemia (major or intermediate) and by type of treatments. The present study aimed to determine the correlation between serum levels of hepcidin and ferritin in patients with thalassemia major and intermedia under chelation therapy. This cohort study investigated 143 patients with thalassemia (122 patients with thalassemia major and 21 patients with and thalassemia intermedia) who referred to Thalassemia Center of Qom University of Medical Sciences, Qom, Iran. The serum level of ferritin was measured twice in all patients: in the beginning and after taking iron chelator by ECL method. Hepcidin was assayed at the end of the study by ELISA methods. Demographic data and clinical history of the patients (type of disease, type of drugs used, and blood transfusion history) were completely recorded. The mean serum level of hepcidin in the TM group (2249.62 ± 1547.37 ng/ml) was greater than the TI group (1482.43 ± 1314.26 ng/ml) (p = 0.007). However, there was no meaningful difference in serum ferritin level between the two groups (2997.74 ± 2545.66 vs. 2670.62 ± 2670.04 ng/ml, p > 0.05). Serum ferritin and hepcidin levels were not correlated both in TM or TI patients (p > 0.05). However, evaluating the trend of changes in serum levels of ferritin with hepcidin showed a significant association. High serum levels of ferritin indicated high iron overload in both groups, TM and TI, which decreased after treatment with chelators. The higher hepcidin levels in TM patients in comparison to TI patients could reflect the higher iron overload in these patients. However, further cellular studies are needed to evaluate the accuracy of these tests in the assessment of iron overload.

Primary plasma cell leukaemia in a 20-year young adult male: a rare presentation

Co-occurrence of CALR and MPL somatic mutations in an Indian patient with a Philadelphia-negative myeloproliferative neoplasm Abstract Philadelphia-negative myeloproliferative neoplasms (MPNs) are a group of clonal disorders that are characterized by excessive proliferation of abnormal myeloid precursors and mature cells. Somatic driver mutations in the JAK2, CALR, and MPL genes serve as major diagnostic criteria in the classification of the MPNs, namely polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Although initially thought to be mutually exclusive, recent studies have reported the co-existence of JAK2, MPL, and CALR mutations. In this case report, we describe a case of a Philadelphia-negative myeloproliferative neoplasm harboring mutations in the CALR [NM_004343.3:c.1092_1143del52 (NP_004334.1:p.Leu367Thrfs)] and MPL [NM_005373.2:c.1543T>A (NP_005364.1:p.Try515Arg)] genes. Given the rarity of documented co-occurrence of driver mutations in these two genes and the concomitant paucity of data regarding management of patients harboring mutations in both these genes simultaneously, there are no clear guidelines for the treatment of patients with these mutation patterns and hence it is difficult to assess the true relevance of this genotype. The fact that both the MPL and CALR genes are a part of the JAK-STAT pathway could, however, assist in the clinical decision-making process.

Inconsistency associated with SOX11 immunohistochemistry in mantle cell lymphoma: a meta-analysis Abstract SOX11 immunohistochemistry (IHC) in mantle cell lymphoma (MCL) is known to show varied results. Our aim was to evaluate the factor responsible for this variation among different studies. A meta-analysis was performed with the original data including the proportion and number of SOX11-positive MCL cases, host and clonality of SOX11 antibodies, clone or catalog number of antibodies, MCL subtypes, number of cases with indolent traits, number of aggressive variants, and cut-off for SOX11 IHC interpretation. A total of 21 published studies were analyzed. The combined proportion of SOX11-positive MCL cases was 0.80 (95% CI = [0.72, 0.87]), and substantial heterogeneity was observed (I2 = 83%). To explore sources of heterogeneity, subgroup analysis and meta-regression were done. Subgroup analysis with moderators of antibody clone or catalog number, antibody clonality, and monoclonal antibody clones showed substantial residual heterogeneity. Meta-regression with moderators of the proportion of cut-off value showed statistically significant result, although that with the aggressive cases did not. However, meta-regression with the cut-off value as a moderator showed substantial heterogeneity. The current meta-analysis of SOX11 immunohistochemistry in MCL showed the cut-off value to be important sources of overall heterogeneity.

Post-transplant lymphoproliferative disorder manifesting as lymphomatoid granulomatosis: report of two cases and review of the literature highlighting current challenges in pathologic classification Abstract Classification of viral- and immunodeficiency-associated lymphoproliferative disorders continues to evolve. Entities in these categories recognized in the 2016 revision of the World Health Organization Classification of Lymphoid Neoplasms show considerable morphologic overlap, and it is unclear whether the current classification conceptualizes the scenarios in which these lesions arise and how they might be best managed. Here, we report two cases of lymphoproliferative disorders meeting histologic criteria for lymphomatoid granulomatosis that arose following solid organ transplant. In reviewing the clinicopathologic features of these examples and those of similar cases in the literature, we highlight challenges in current classification and opportunities for their refinement.

Determining the frequency of iron overload at diagnosis in de novo acute myeloid leukemia patients with multilineage dysplasia or myelodysplasia-related changes: a case control study Abstract Acute myeloid leukemia (AML) with myelodysplasia-related changes (AML-MRC) is a new disease category, which was defined as a separate entity in the World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. While pre-treatment iron overload in patients with myelodysplastic syndrome has been previously studied, its relationship with AML-MRC has not been studied. We aimed to investigate the relationship between serum iron tests compatible with iron overload and the diagnosis of multilineage dysplasia (MLD) and AML with myelodysplasia-related changes (AML-MRC) in AML patients diagnosed at Hacettepe University Adult Hospital between January 2002 and September 2017. Ninety-three patients who met the criteria were enrolled. Bone marrow aspirate of each patient was re-examined, and dysplasia was investigated; other data were examined from patient’s records. The iron overload status at diagnosis and transferrin saturation (TS) values were compared between the groups with and without MLD and those with and without AML-MRC. When iron overload was defined as TS ≥ 58% and ferritin ≥ 500 ng/mL, iron overload was observed in 10 (37%) patients with MLD and in 4 (13%) without MLD. The difference is almost statistically significant (p = 0.053). The mean TS value and frequency of iron overload were higher in AML-MRC patients than in non-AML-MRC patients (p < 0.05 for both). A mild positive significant correlation was observed between the dysplasia severity score and TS (r = 0.317, p = 0.032). In patients with AML-MLD and AML-MRC, iron overload occurred regardless of the transfusion status at the time of diagnosis. Morphologic severity of dysplasia may be correlated with higher TS values at the time of diagnosis.

Systemic mastocytosis with renal light chain amyloidosis: associated non-mast cell disorder or concurrent disease Abstract Systemic mastocytosis with an associated hematological neoplasm (SM-AHN) is a rare diagnosis commonly associated with myeloid disorders. Herein, we describe the case of a 53-year-old female diagnosed with systemic mastocytosis (SM) in conjunction with renal light chain amyloidosis (AL) and smoldering myeloma. Although cytokines such as IL-6 may play a role in the proliferation of plasma cells, delays in the diagnosis of SM and paucity of relevant studies cast uncertainty on whether the associated disease is secondary to the mast cells or etiologically independent. To our knowledge, this is the first case of confirmed renal AL disease in conjunction with SM. We review and summarize the available literature describing SM in association with plasma cell disorders.