Thrombocytopenia as the Initial Presentation of

Thrombocytopenia as the Initial Presentation
of Angioimmunioblastic T-Cell Lymphoma (AITL)
Maria Batool, MD; Biana Leybishkis, MD; Nihad Yasmin, MD
Aurora Health Care Internal Medicine Residency – Milwaukee, WI
Case Presentation:
Further Workup:
• 85 year old female presented with a one week history of
• Bone marrow biopsy:
generalized weakness and knee pain.
• PMH: HTN, GERD, osteoarthritis.
• SH: No history of alcohol, tobacco or illicit drug use.
• Review of symptoms was negative except for dry cough.
Physical Examination:
– Moderate normocytic anemia, severe thrombocytopenia
and mild immature myeloid shift
• Serum electrophoresis:
– Polyclonal elevation of gamma globulins without
monoclonal protein
Discussion:
•A
ITL is a type of peripheral T-cell non-Hodgkin lymphoma.
•M
ost commonly presents with systemic B symptoms, generalized
lymphadenopathy, hepatosplenomegaly, polyarthritis and anemia.
•9
1% of patients with AITL have involvement of at least 2 or more lymph
node groups palpable on physical exam which was absent in our patient.
•7
% of patients have idiopathic thrombocytopenic purpura which is a
• Right axillary lymph node biopsy:
consequence of the immune dysregulation that is part of the spectrum
of AITL.
– Pathology diagnostic for Angioimmunoblastic T-cell Lymphoma
• Bone marrow is infiltrated in 60% of the patients.
•V
ital signs: BP: 112/52, Pulse: 91/min, RR:16, Temp: 99,
•S
tudies looking specifically at patients with uninvolved bone marrow
Pulse Ox: 100% on room air.
found only one of six cases to have platelet count below 150,000 x 109
which was actually the only significant initial finding in our patient.
• HEENT: mild conjunctival pallor.
• Musculoskeletal: 1+ pitting edema bilateral lower extremities.
• Lymphatics: No peripheral lymphadenopathy.
Conclusion:
•A
ITL presents a diagnostic challenge for physicians because of its
Laboratory Data:
nonspecific hematologic and immunologic manifestations.
• Hemoglobin: 9.2 g/dl
• Platelets: 10,000 x 109/L
• HCT: 29.2%
• INR: 1.5
• MCV: 82fl
• Adams 13: Negative
• Peripheral smear: Schistocytes
• LDH: 412 U/L
•A
ITL must be kept in mind in the setting of unexplained
thrombocytopenia even if the typical features of a lymphoma are absent.
CISH staining for EBV positive cells.
• Absolute reticulocyte count: 51 x 109/L
References:
1. A
ngioimmunoblastic T-Cell lymphoma: a critical analysis of clinical,
morphologic and immunophenotypic features. Bal M. Gujral
Imaging:
S. Gandhi J. Shet T. Epari S. Subramanian PG. Indian Journal of
Pathology & Microbiology. 53(4):640-5, 2010 Oct-Dec.
CT abdomen showed extensive retroperitoneal, pelvic, inguinal and
2. E valuation and management of angioimmunoblastic T-cell
mesenteric lymphadenopathy.
lymphoma: a review of current approaches and future strategies.
[Review] [100 refs] Alizadeh AA. Advani RH. Clinical Advances in
Hematology & Oncology. 6(12):899-909, 2008 Dec.
3. A
ngioimmunoblastic T-cell lymphoma. [Review] [57 refs] Iannitto
E. Ferreri AJ. Minardi V. Tripodo C. Kreipe HH. Critical Reviews in
Oncology-Hematology. 68(3):264-71, 2008 Dec.
4. A
ngioimmunoblastic T-cell lymphoma: clinical and laboratory
Immunohitochemical stain for CD 3, a T-cell marker.
features at diagnosis in 77 patients. Lachenal F. Berger F. Ghesquieres
H. Biron P. Hot A. Callet-Bauchu E. Chassagne C. Coiffier B. Durieu I.
Rousset H. Salles G. Medicine. 86(5):282-92, 2007 Sep.
5. C
linical features and treatment outcomes of angioimmunoblastic
Clinical Course:
T-cell lymphoma. Park BB. Ryoo BY. Lee JH. Kwon HC. Yang SH. Kang
Platelets and hemoglobin levels continued to fall. Patient subsequently
Kang JH. Bang SM. Park S. Kim K. Park K. Suh C. Kim WS. Leukemia
received intravenous immunoglobulin, high dose dexamethasone,
& Lymphoma. 48(4):716-22, 2007 Apr.
platelets and blood transfusions.
HJ. Kim HJ. Oh SY. Ko YH. Huh JR. Lee SS. Nam EM. Park KW. Kim JH.