PMC5399210_01
Splenic bleeding of splenic marginal zone lymphoma (SMZL). (A) A dynamic CT scan of the abdomen on admission. (B) Histology of the excised spleen. Hematoxylin and Eosin (H&E) staining. Magnification, x100, insetx1,000. (C) Histology of the excised lymph node. H&E staining. Magnification, x20. Inset, x400. (D) PCR amplification of complementarity determining region-3 (CDR-3). The spleen (Sp) and lymph node (LN) lanes show monoclonal bands of the same size. VH26, forward primer. VLJH, reverse primer for second PCR. JH, reverse primer for first PCR. M, size marker. Neg, negative control. Pos, positive control.
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Dynamic CT revealed even larger splenomegaly with active bleeding and multiple intraabdominal lymph nodes (mesenteric and paraarortic) that were increased in number and size compared to those in 2007 (Fig. 1A). Histology revealed massive bleeding in the form of peliosis (Fig. 1B). In addition, there were clusters of atypical lymphocytes (Fig. 1B, inset). The pathological findings showed monotonous growth of atypical lymphocytes similar to that in the excised spleen (Fig. 1C). The PCR products of CDR-3 using tissue from the spleen and a lymph node were of the same size, and their sequences matched perfectly, indicating that the spleen and lymph nodes had lymphoma cells of the same origin (Fig. 1D).
Staging of SMZL. (A) Gastroendoscopy image. Mucosal biopsy revealed lymphoma invasion (data not shown). (B) A bone marrow smear. The black arrows show lymphoma cells. Wright-Giemsa staining. Magnification, x1,000. (C) Colonoscopy image. The mucosae of the sigmoid colon and rectum were mildly opaque. (D) A contrast-enhanced CT scan of the lungs. Pleural effusion and atelectasis of the left lower lung are shown. (E) A contrast-enhanced CT scan of the abdomen. Arrowheads, swollen lymph nodes.
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Bence Jones protein kappa-type (data not shown). Biopsy of the gastric mucosa and bone marrow aspiration/biopsy revealed the invasion of lymphoma cells (Fig. 2A and B and data not shown). Intestinal invasion was also suspected by colonoscopy (Fig. 2C). CT showed left pleural effusion and atelectasis of the left lower lobe, suggesting pleural infiltration of lymphoma (Fig. 2D) and multiple intraabdominal lymphadenopathies (Fig. 2E).
Coagulopathy due to AVWS. (A) - (C) Decreased ristocetin-induced platelet aggregation. Platelet aggregation curves induced by (A) ristocetin, (B) ADP, and (C) collagen are shown. The blue, green, and red lines show platelet aggregation of small, medium, and large size, respectively (left axis). The black line shows the total aggregation (right axis). The aggregation was positive when the total coagulation was higher than 50%. (D) von Willebrand factor multimer assay. The absence of large- and medium-sized multimers is shown.
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ADP and collagen was normal (Fig. 3A-C). A VWF multimer assay showed an absence of large- and medium-sized multimers, similar to type 2A VWD (Fig. 3D). A cross-mixing test with normal plasma compensated the VWF activity to 33% (0 h incubation) and 39% (2 h incubation), ruling out the presence of anti-VWF inhibitors (Table 2).