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Slide Seminar 01 - Peripheral T-cell and NK-cell Lymphomas
Sunday, September 17, 2006 08:00 - 12:00
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Moderators:
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Dr. Elaine S. Jaffe and Dr. Philippe Gaulard
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Disclosure:
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In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Slide Seminar) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. Faculty members for this Slide Seminar have indicated they have no disclosures to make.
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Clinical histories are displayed below.
Click on the case numbers to display the text and references for each case.
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- Introduction to Peripheral T-cell Lymphomas and Angioimmunoblastic T-cell Lymphoma
Submitted by: Dr. Elaine S. Jaffe
The patient is a 60 year old male with fever, skin rash, thrombocytopenia, leukocytosis, and generalized lymphadenopathy. The patient was found to have a monoclonal IgM spike in serum and urine, with plasma cell infiltrates in bone marrow and a presumptive diagnosis of Waldenstrom's macroglobulinemia. The patient was treated with high dose steroids, had transient response and the symptoms recurred. A lymph node biopsy was obtained.
Case 1 - Figure 1
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- Peripheral T-cell Lymphoma, Unspecified (PTCL-U)
Submitted by: Dr. H.K. Muller-Hermelink
63 year old female with a single enlarged lymph node, present for four months. Questionable infection and distinct arthritis.
Case 2 - Figure 1
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- Primary Gastric T-cell Lymphoma associated with Human T-cell Leukemia Virus Type I
Submitted by: Tadashi Yoshino, MD, PhD
A 44-year-old female, who lived outside the ATL-endemic, southwestern, area of Japan, consulted her physician about abdominal discomfort and was hospitalized for chronic cholecystolithiasis. During her hospitalization, a gastric ulcer was detected. An endoscopic examination revealed an irregular-shaped ulcer at the posterior wall of the upper portion of the corpus. Histology of biopsy specimens taken from this lesion disclosed medium-sized lymphoid cells infiltrating and destroying glands. In some areas, lymphoid cells infiltrated into glands, and epithelial elements with intermingled lymphoid cells looked very similar to the lymphoepithelial lesions (LEL) of gastric MALT lymphoma. The destroyed gastric glands were easily detected by immunostaining for keratin. A tentative diagnosis of MALT lymphoma was returned to the clinician, and H. pylori was eradicated. H. pylori disappeared within a short period without improving the ulcer.
The second endoscopic examination, performed three months later, revealed that her gastric lesion had worsened. The biopsied specimens at this time showed no LEL, and immunohistological examination indicated that the infiltrating lymphoid cells were CD3+, CD4+, CD79a-, and CD8-. This finding strongly indicated that the patient had T-cell lymphoma. She was hospitalized again, and a scanning CT examination detected mild swelling of the paragastric lymph nodes, but there were no hepatosplenic or other abdominal organ abnormalities. Her heart and lungs showed no particular findings. The results of laboratory examination were as follows: RBC, 482x100000/ ul; WBC, 7600/ ul; platelets, 30.1x100000/ l; GOT, 16 IU/l; GPT, 16 IU/l; LDH, 155 IU/l (normal 230-490 IU/l); gammaGTP, 14 IU/l; total bilirubin, 0.39 mg/dl; BUN, 10.7 mg/dl; creatinine, 0.64 mg/dl; CPK, 40 IU/l; amylase, 210 IU/l; total protein, 6.0 g/dl; albumin, 3.6 g/dl; Na, 140.6 mEq/l; K, 4.0 mEq/l; Cl, 106 mEq/l; Ca, 8.3 mg/dl; total cholesterol, 182 mg/ dl. Abnormal lymphocytes were not evident. The titer of antibodies against HTLV-I was 20x. The clinical stage of the patient was estimated at IIE, and her entire stomach was surgically resected.
Case 3 - Figure 1
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- T and NK Cell Lymphomas
Submitted by: Dr. Philippe Gaulard
A 54-year-old man presented with fatigue, fever and weight loss revealing a major splenomegaly and hepatomegaly, without lymphadenopathy. He had pancytopenia (WC: 1,4: Hb: 7,7; Plt:25) without abnormal circulating cells. The bone marrow biopsy was hypercellular and first interpreted as normal (*).
Clinical staging and CT scan: no lymphadenopathy
Splenectomy for diagnosis (slide submitted).
Aggressive clinical course, despite intensive polychemotherapy. Dead of disease within ten months after diagnosis.
(*) Retrospective review of the initial bone marrow performed in another institution, disclosed minimal lymphoid infiltration . The bone marrow features will be discussed.
Case 4 - Figure 1
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- Anaplastic Large Cell Lymphoma: After Twenty Years the Controversy Continues
Submitted by: Dr. Marsha Kinney
17 year-old white male with one-month history of adenopathy, fever (103.5 °F), pharyngitis, hepatosplenomegaly, and weight loss of 25 pounds over three months. On admission to a local hospital, the patient was found to have a white blood count of 45,000 uL with 58% lymphocytes, some atypical. Liver function tests were mildly elevated. The patient developed a maculopapular, generalized rash. CT examination revealed bilateral pleural effusions and a possible abscess around the gallbladder. The patient was taken to laparotomy, which showed no abscess. A liver biopsy revealed a polymorphic, periportal, lymphocytic infiltrate. Clinical diagnosis was hepatitis. Monospot was negative. A screen for hepatitis A, B, and C was negative. CMV serum titer was positive at 1:160 for IgM. The patient developed progressive respiratory distress, a worsening rash, and ascites.
The patient was transferred to a tertiary care hospital. On physical examination, his temperature was 105.8 °F. White blood count was 48,300 uL with 47% lymphocytes and 14% monocytes. SGOT was 251 IU/L and LDH of 799 IU/L and alkaline phosphatase of 206 IU/L and total billirubin of 5.8 mg/dl.
An axillary lymph node biopsy was performed and is available for review.
Case 5 - Figure 1
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- CD4+/ CD56+ Tumors and the TCL-1 Oncogene
Submitted by: Dan Jones, MD, PhD
74-year-old white male, who presented in February 2002 with an indurated skin plaque on the right shoulder, which was excised and then recurred 6 months later at the same site. Bone marrow was normocellular with mild monocytosis (10%). Patient received hyperCVAD chemotherapy and has not had a skin recurrence.
Over the last four years, however, the patient has had an increasingly hypercellular bone marrow (60-80% cellular) with marrow monocytosis (up to 23%) and absolute PB monocytosis up to 2.5 x 10(9)/L.
Case 6 - Figure 1
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