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HIV-Heme/Onc
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Case 31-2003: A 44-Year-Old Man with HIV Infection and a Right Atrial Mass (Diffuse Large B Cell Lymphoma)
AIDS-related lymphoproliferative disease Blood 2006



Case Discussion:
25 yo AAM with HIV and CD4 of 350 is seen for back pain. Shows bony destruction of the L2 vertebrae with lytic changes. Multiple bony lytic lesion is also seen. Has diffuse lymphadenopathy as well. Prominent bilateral axillary, left paratracheal, pericardial and internal mammary chain lymph nodes; Multiple enlarged lymph nodes throughout the retroperitoneum and mesentery. Multiple prominent inguinal lymph node is present as well. Patient continues to have daily fever. Patient was diagnosed of HIV in 2008, and took medication for few years. He stopped taking medications and only recently in last 2 month was patient started on HIV medication. CD4 count at this time was 350.
Other lab. 
Alb : Low; NO Urine Albumin was done. 
Increased Globulin. Normal SPEP, S IFA increased IgG, Normal FLC 
ALP increase.
CRP, ESR increased. 
Decreased Testesterone, but normal hcg
RPR reactive, FTA-ABS reactive.
WBC 15, Platelet 628, Hgb 8.5 
Inguinal Lymph Node Biopsy is done. What is the presumptive diagnosis at this time. 
Small bilateral Pleural effusion is also noted. Spleen and Liver are not enlarged. 

Approach to the Lytic Lesoin

http://www.medscape.com/viewarticle/477692_3

Age is the most important factor to narrow the DDx

  • metastatic neuroblastoma in the infant and young child, 
  • metastasis and multiple myeloma in the middle-aged and elderly,
  • Ewing's sarcoma and simple bone cyst in the long bones in children and young teenagers, and 
  • giant cell tumor in the young to middle-aged adult (20 to 50 years of age)
The next step is to examine the lesion to see if it has a pathognomonic appearance and/or location. Some lytic lesions have a characteristic radiographic appearance (including matrix) and/or location that are inherently diagnostic. 
  • A few examples include: a corduroy vertebral body (hemangioma), 
  • a fallen fragment sign (simple bone cyst; ), 
  • intralesional gas in a juxta-articular lesion (subchondral cyst, such as a degenerative cyst or intraosseous ganglion cyst;), 
  • an enlarged bone with coarsened trabecular and a thickened cortex (Paget's disease), 
  • chondroid matrix in a geographic lytic lesion in the hand (enchondroma; ),
  •  vertebra plans in an otherwise healthy child (Langerhan's cell histiocytosis), and 
  • the cockade sign in the calcaneus (intraosseous lipoma).

DDx for Lymphadenopathy

Cancer

  • Non‐Hodgkin’s lymphoma  ( Is possible..)
  • Hodgkin’s lymphoma  ( Is possible..)
  • Kaposi’s sarcoma (CD 4 count is high -- less likely) 
  • Castleman’s disease  ( Is possible..)
    • Fever – 100 percent (present in this patient)
    • Lymphadenopathy – 96 percent (present in this patient)
    • Splenomegaly – 86 percent (not present in this patient)
    • Hepatomegaly – 63 percent (not present in this patient)
    • Pulmonary signs or symptoms – 35 percent
    • Edema – 29 percent (not present in this patient)
    • Ascites – 6 percent (not present in this patient)
    • Elevated CRP is seen (IL 6), Pulm findings are not uncommon. Pleural effusion can occur. 
    • Can occur as a part of POEMS in which S stands for skin changes. 
    • Can have SCLEROTIC Bone lesion. Our patient has Lytic Bony Lesion
  • Anal cancer Metastatic carcinoma ( Is possible..)
  • Genital cancer (nothing is seen in examination..less likely)
  • Melanoma  ( Is possible..)
  • Neuroendocrine tumor (Merkel‐cell carcinoma)  ( Is possible..)

Infection

Bacterial Bartonellosis

  • Syphilis  ( Is possible.., but typically should not cause lytic lesion)
  • Lymphogranuloma venereum (less likely due to lytic lesion and diffuse lymphadenopathy
  • Chancroid (less likely due to lytic lesion and diffuse lymphadenopathy
  • Tularemia (less likely due to lytic lesion and diffuse lymphadenopathy
  • Yersinia pestis infection (less likely due to lytic lesion and diffuse lymphadenopathy
  • Staphylococcus aureus infection (less likely due to lytic lesion and diffuse lymphadenopathy
  • Streptococcal infection 
  • Brucellosis
  • Mycobacterial and diffuse lymphadenopathy
  • Mycobacterium tuberculosis infection  (less likely due to lytic lesion
  • Nontuberculous mycobacterial infection (less likely due to lytic lesion

Viral

  • Herpes simplex virus  (less likely due to lytic lesion
  • Epstein–Barr virus Cytomegalovirus (less likely due to lytic lesion
  • Human immunodeficiency virus (less likely due to lytic lesion, and diffuse enlargement of the lymph node)

Fungal

  • Cryptococcosis Histoplasmosis Blastomycosis Coccidioidomycosis Sporotrichosis

Protozoal (toxoplasmosis)

Other causes

  • Immune reconstitution inflammatory syndrome 
  • Sarcoidosis
  • Drug‐related hypersensitivity
  • Autoimmune disease (systemic lupus erythematosus) Kikuchi’s disease (histiocytic necrotizing lymphadenitis) Kimura’s disease
  • Rosai–Dorfman disease (sinus histiocytosis) 
Thus diseases causing lytic lesions and lymphadenopathy are
1) Baciallary angiomatosis.(usually low CD4)
2) Kaposi Sacroma (usually Low CD4)
3) Multi centric Castle man disease (usually sclerotic bone lesion is seen ??? IL6 causes lytic lesions mostly) 
4) Metastatic Anal Cell Cancer (Less likely due to pattern of lymphonode in groin, and chest, not typical for metastatic anal cell cancer)
5) NHL (HD causes osteoblastic disease)
6) Syphilis (osteolytis is a rare presentation)
7) Plasmablastic Lymphoma

Priortizing DDx
1) Plasmablastic Lymphoma (like MM but M spike is not present; typically in advanced HIV; 
2) NHL 
3) 

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